Geriatric Clinical Advisor: Instant Diagnosis and Treatment

1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899

GERIATRIC CLINICAL ADVISOR: INSTANT DIAGNOSIS AND TREATMENT

ISBN-13: 978-0-323-04195-9 ISBN-10: 0-323-04195-7

Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Permissions may be sought directly from Elsevier's Health Sciences Rights Department in Philadelphia, PA, USA: phone: (+1) 215 239 3804, fax: (+1) 215 239 3805, e-mail: [email protected]. You may also complete your request on-line via the Elsevier homepage (http://www.elsevier.com), by selecting “Customer Support” and then “Obtaining Permissions.”

Notice Knowledge and best practice in this field are constantly changing. As new research and experience broaden our knowledge, changes in practice, treatment, and drug therapy may become necessary or appropriate. Readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on his or her own experience and knowledge of the patient, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the Editor assumes any liability for any injury and/or damage to persons or property arising out of or related to any use of the material contained in this book. The Publisher International Standard Book Number 0-323-04195-7 International Standard Book Number (PDA online) 0-323-04356-9

Acquisitions Editor: Rolla Couchman Developmental Editor: Mary Beth Murphy

Printed in the United States of America Last digit is the print number: 9 8 7

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The editors would like to dedicate this book to their families and friends.

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Associate Editors FRED F. FERRI, M.D., F.A.C.P.

JOHN B. MURPHY, M.D.

Clinical Professor Department of Community Health Brown Medical School Providence, Rhode Island

Division of Geriatrics Rhode Island Hospital Providence, Rhode Island

SECTION I: DISEASES AND DISORDERS, SECTION II: DIFFERENTIAL DIAGNOSIS, SECTION III: CLINICAL ALGORITHMS, SECTION IV: LABORATORY TESTS AND INTERPRETATION OF RESULTS

APPENDIX 1: SYSTEMS OF GERIATRIC CARE

AMAN NANDA, M.D., C.M.D. Assistant Professor in Medicine Brown Medical School Providence, Rhode Island APPENDIX 2: TOOLS OF GERIATRIC ASSESSMENT

CYNTHIA HOLZER, M.D, C.M.D. Director, Geriatric Education Roger Williams Medical Center Providence, Rhode Island Assistant Professor of Medicine Boston University School of Medicine Boston, Massachusetts

TOM J. WACHTEL, M.D., F.A.C.P.

SECTION V: CLINICAL PREVENTIVE SERVICES

SECTION I: DISEASES AND DISORDERS, SECTION II: DIFFERENTIAL DIAGNOSIS, SECTION III: CLINICAL ALGORITHMS, SECTION IV: LABORATORY TESTS AND INTERPRETATION OF RESULTS, APPENDIX 1: SYSTEMS OF GERIATRIC CARE

LYNN MCNICOLL, M.D. Division of Geriatrics Rhode Island Hospital Providence, Rhode Island

Division of Geriatrics Rhode Island Hospital Professor of Community Health and Medicine Brown Medical School Providence, Rhode Island

SECTION I: DISEASES AND DISORDERS

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Contributors PHILIP J. ALIOTTA, M.D., M.S.H.A., F.A.C.S.

JOHN E. CROOM, M.D., PH.D.

Clinical Instructor Department of Urology School of Medicine and Biomedical Sciences State University of New York at Buffalo Buffalo, New York Medical Director Center for Urologic Research of Western New York Williamsville, New York

Clinical Fellow in Neurology Harvard Medical School Beth Israel Deaconess Medical Center Boston, Massachusetts

GEORGE O. ALONSO, M.D. Director Department of Infection Control Elmhurst Hospital Center Elmhurst, New York Instructor in Medicine Mount Sinai School of Medicine New York, New York

MEL L. ANDERSON, M.D., F.A.C.P. Clinical Assistant Professor of Medicine Brown Medical School Providence, Rhode Island

PATRICIA AREAN, PH.D. Associate Professor Department of Psychiatry University of California, San Francisco San Francisco, California

MICHAEL G. BENATAR, M.B.CH.B., D.PHIL. Assistant Professor of Neurology Department of Neurology Emory University Atlanta, Georgia

CLAUDIA L. DADE, M.D. Attending Physician Division of Infectious Diseases Elmhurst Hospital Center Elmhurst, New York Instructor in Medicine Mount Sinai School of Medicine New York, New York

GEORGE T. DANAKAS, M.D., F.A.C.O.G. Clinical Assistant Professor Department of Obstetrics and Gynecology State University of New York at Buffalo Buffalo, New York

AMAR DESAI, M.D., M.P.H. Resident Physician Department of Internal Medicine University of California, San Francisco San Francisco, California

JEFFREY S. DURMER, M.D., PH.D. Assistant Professor, Department of Neurology Director, Emory Sleep Laboratory Director, Egleston Children’s Hospital Sleep Clinic Emory University School of Medicine Atlanta, Georgia

SUSAN BERNER, M.D. Geriatrics Fellow, Department of Family Medicine University of Cincinnati College of Medicine Cincinnati, Ohio

LYNN BOWLBY, M.D. Attending Physician Division of General Internal Medicine Rhode Island Hospital Clinical Instructor of Medicine Brown Medical School Providence, Rhode Island

MARIA A. CORIGLIANO, M.D., F.A.C.O.G. Clinical Assistant Professor Department of Obstetrics and Gynecology State University of New York at Buffalo Buffalo, New York

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GARY EPSTEIN-LUBOW, M.D. Postdoctoral Research Fellow Department of Psychiatry and Human Behavior Brown Medical School Staff Psychiatrist Butler Hospital Providence, Rhode Island

MARK J. FAGAN, M.D. Director Medical Primary Care Unit Rhode Island Hospital Associate Professor of Medicine Brown Medical School Providence, Rhode Island

STEPHEN CORREIA, PH.D.

GIL M. FARKASH, M.D.

Dementia Research Fellow Department of Psychiatry and Human Behavior Brown Medical School Providence, Rhode Island

Assistant Clinical Professor State University of New York at Buffalo School of Medicine Buffalo, New York

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Contributors MITCHELL D. FELDMAN, M.D., M.PHIL.

CYNTHIA HOLZER, M.D., C.M.D.

Associate Professor of Clinical Medicine University of California, San Francisco Division of General Internal Medicine San Francisco, California

Director, Geriatric Education Roger Williams Medical Center Providence, Rhode Island Assistant Professor of Medicine Boston University School of Medicine Boston, Massachusetts

FRED F. FERRI, M.D., F.A.C.P. Clinical Professor Department of Community Health Brown Medical School Providence, Rhode Island

GLENN G. FORT, M.D., M.P.H. Clinical Associate Professor of Medicine Brown Medical School Chief Infectious Diseases Our Lady of Fatima Hospital North Providence, Rhode Island

MICHAEL P. GERARDO, D.O., M.P.H. Post-Doctoral Research Fellow Brown Medical School, Center for Gerontology and Health Care Research Brown University Geriatric Medicine Fellow Rhode Island and Miriam Hospitals Providence, Rhode Island

SUSAN E. HARDY, M.D., PH.D. Assistant Professor Geriatric Medicine University of Pittsburgh School of Medicine Pittsburgh, Pennsylvania

MIKE HARPER, M.D. Associate Professor of Medicine Division of Geriatrics Department of Medicine San Francisco Veterans Affairs Medical Center University of California, San Diego San Diego, California

JASON IANNUCCILLI, M.D. Department of Medicine Brown Medical School Providence, Rhode Island

RICHARD S. ISAACSON, M.D. Director, Research Unit in Medical Education Associate Medical Director Wien Center for Memory Disorders and Alzheimer’s Disease Assistant Professor of Medicine University of Miami—Leonard M. Miller School of Medicine Departments of Medical Education, Internal Medicine, and Neurology Mount Sinai Medical Center Miami Beach, Florida

BREE JOHNSTON, M.D., M.P.H. Associate Professor of Medicine Division of Geriatrics Department of Medicine Veterans Affairs Medical Center University of California, San Diego San Diego, California

MELVYN KOBY, M.D. Associate Clinical Professor of Medicine Department of Ophthalmology University of Louisville School of Medicine Louisville, Kentucky

E. GORDON MARGOLIN, M.D. Professor, Internal Medicine and Geriatrician University of Cincinnati College of Medicine Cincinnati, Ohio

TAYLOR HARRISON, M.D.

DANIEL T. MATTSON, M.D., M.S.C.(MED.)

Neuromuscular Fellow Department of Neurology Emory University Atlanta, Georgia

Clinical Fellow in Neurology Beth Israel Deaconess Medical Center Harvard Medical School Boston, Massachusetts

SHARON S. HARTMAN, M.D., PH.D.

LYNN MCNICOLL, M.D.

Clinical Associate Department of Neurology Emory University Atlanta, Georgia

Division of Geriatrics Rhode Island Hospital Providence, Rhode Island

HOLLY M. HOLMES, M.D.

Clinical Instructor Department of Orthopedic Surgery Creighton University School of Medicine Omaha, Nebraska

Instructor University of Chicago Chicago, Illinois

LONNIE R. MERCIER, M.D.

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Contributors DENNIS J. MIKOLICH, M.D., F.A.C.P., F.C.C.P.

SEAN I. SAVITZ, M.D.

Chief Division of Infectious Diseases VA Medical Center Clinical Associate Professor of Medicine Brown Medical School Providence, Rhode Island

Clinical Fellow in Neurology Harvard Medical School Chief Resident in Neurology Beth Israel Deaconess Medical Center Boston, Massachusetts

RAM R. MILLER, M.D.C.M., M.S., F.R.C.P.C.

Assistant Professor, Division of Hematology/Oncology Northwestern University The Feinberg School of Medicine Chicago, Illinois

Assistant Professor University of Maryland School of Medicine Division of Gerontology Department of Epidemiology and Preventive Medicine Baltimore, Maryland

ARVIND MODAWAL, M.D., M.P.H., M.R.C.G.P. Associate Professor Department of Family Medicine, Section of Geriatrics University of Cincinnati College of Medicine Cincinnati, Ohio

JOHN B. MURPHY, M.D. Division of Geriatrics Rhode Island Hospital Providence, Rhode Island

AMAN NANDA, M.D., C.M.D. Assistant Professor in Medicine Brown Medical School Providence, Rhode Island

TAKUMA NEMOTO, M.D. Research Associate Professor of Surgery State University of New York at Buffalo Buffalo, New York

JOSEPH W. SHEGA, M.D.

CLIFFORD MILO SINGER, M.D. Associate Professor of Psychiatry Director, Insomnia and Chronobiology Clinic Vermont Regional Sleep Center University of Vermont College of Medicine Burlington, Vermont

U. SHIVRAJ SOHUR, M.D., PH.D. Clinical Fellow in Neurology Harvard Medical School Chief Resident in Neurology Beth Israel Deaconess Medical Center Boston, Massachusetts

JOHN A. STOUKIDES, M.D., R.PH. Director of Geriatrics Roger Williams Medical Center Providence, Rhode Island Clinical Assistant Professor of Medicine Boston University School of Medicine Boston, Massachusetts Adjunct Associate Professor of Pharmacy and Nursing University of Rhode Island Kingston, Rhode Island

PRANAV M. PATEL, M.D.

JULIE ANNE SZUMIGALA, M.D.

Clinical Associate Instructor, Division of Cardiology Department of Medicine Brown Medical School Providence, Rhode Island

Clinical Instructor Department of Obstetrics and Gynecology State University of New York at Buffalo Buffalo, New York

PETER PETROPOULOS, M.D.

LAURA TRICE, M.D.

Department of Veterans Affairs Providence, Rhode Island

Medical Director Trihealth SeniorLink California, Kentucky

ARUNDATHI G. PRASAD, M.D.

EROBOGHENE E. UBOGU, M.D.

Clinical Instructor Department of Obstetrics and Gynecology/Resident Education State University of New York at Buffalo Women’s and Children’s Hospital Buffalo, New York

Assistant Professor of Neurology Case Western Reserve University School of Medicine Staff Neurologist Louis Stokes Cleveland Veterans Affairs Medical Center Cleveland, Ohio

HARLAN G. RICH, M.D.

TOM J. WACHTEL, M.D., F.A.C.P.

Director of Endoscopy Rhode Island Hospital Associate Professor of Medicine Brown Medical School Providence, Rhode Island

Division of Geriatrics Rhode Island Hospital Professor of Community Health and Medicine Brown Medical School Providence, Rhode Island

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Contributors WEN-CHIH WU, M.D.

MADHAVI YERNENI, M.D.

Assistant Professor of Medicine Brown Medical School Cardiologist Providence VA Medical Center Providence, Rhode Island

Staff Physician, Academic Medical Center Miriam Hospital Pawtucket, Rhode Island

BETH J. WUTZ, M.D.

Fellow, Movement Disorders Morton and Gloria Shulman Movement Disorders Center Toronto Western Hospital Toronto, Ontario

Clinical Assistant Professor of Medicine Division of Internal Medicine/Pediatrics Kajeida Health–Buffalo General Hospital State University of New York at Buffalo Buffalo, New York

CINDY ZADIKOFF, M.D.

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Abuse, Elder BASIC INFORMATION

SYNONYMS Geriatric abuse Battered elder syndrome ICD-9CM CODES 995.81 Adult maltreatment syndrome

EPIDEMIOLOGY & DEMOGRAPHICS U.S. INCIDENCE & PREVALENCE: Unknown, statistics are thought to underreport the problem significantly. There were estimated to be 1 million victims of elder abuse in 1996; if self-neglect is included, that number increases to more than 2 million (National Center on Elder Abuse). • Some experts estimate that only 1 of 14 abuse incidents is reported. • In 1996 there were about 300,000 reports of domestic elder abuse.

CLINICAL PRESENTATION • Physical abuse with multiple injuries at various stages with implausible or inconsistent descriptions of their origins; injuries are usually to head, neck, chest, breast, and abdomen. • Extreme fear, hypervigilance, or withdrawal. • Evidence of poor nutrition or hygiene. • Toxicologic evidence of unprescribed medications. • Poor adherence, frequent no-shows.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Advancing dementia • Depression or other psychiatric disorders • Malnutrition due to intrinsic causes • Conscious nonadherence • Financial hardship • Falling • Diogenes syndrome

WORKUP • Interview patient separately from the suspected abuser. • Build trust; patients may be reticent. • Ask direct questions. • Be aware that physical findings are usually unexplained injuries or burns. • Pelvic examination if sexual abuse suspected.

LABORATORY TESTS • If sexual abuse suspected, screening for sexually transmitted diseases • Toxicology screens or therapeutic drug monitoring

IMAGING STUDIES X-rays as indicated by physical presentation

NONPHARMACOLOGIC THERAPY Reporting abuse to Adult Protective Services is mandatory in most states. This also provides the physician access to specialized personnel who can aid in evaluation and disposition. • Separate patient and abuser. • If the burden of care appears to be the major factor in contributing to abuse, referral to respite services, if available, can be useful. Patient and caregiver may benefit from screening and treatment for depression, substance abuse, anxiety, mental illness, or cognitive impairment.

ACUTE GENERAL Rx As indicated for injury or pain relief

DISPOSITION In emergencies, hospitalization may be required. If the patient’s level of disability does not allow for independent living, institutionalization may be required. Guidelines vary at the state and county levels regarding guardianship and conservatorship requirements.

REFERRAL Adult Protective Services (mandatory in most states)

PEARLS & CONSIDERATIONS COMMENTS Consider home visit if abuse, neglect, or self-neglect is suspected.

PREVENTION • Screen for caregiver stress. • Respite services, if possible. • Make financial arrangements and arrange durable power of attorney for health care and finances while patient still cognitively intact.

PATIENT/FAMILY EDUCATION Alzheimer’s Association for caregivers of patients with dementia

SUGGESTED READINGS Fulmer T et al: Progress in elder abuse screening and assessment instruments, J Am Geriatr Soc 52(2):297, 2004. Jogerst GJ et al: Domestic elder abuse and the law, Am J Public Health 93:2131, 2003. Levine JM: Elder neglect and abuse. A primer for primary care physicians, Geriatrics 58(10):37,42, 2003. National Center on Elder Abuse: http://www. elderabusecenter.org. AUTHORS: BREE JOHNSTON, M.D., M.P.H., and MIKE HARPER, M.D.

DISEASES AND DISORDERS

Elder abuse is the willful infliction of physical pain or injury; emotional pain, injury, humiliation, or intimidation; exploitation or misappropriation of money or property; or neglect by the designated caregiver. In general, three basic categories of elder abuse exist: domestic elder abuse (or abuse in the home); institutional elder abuse (abuse that occurs in nursing homes, foster homes, group homes, board and care facilities); and self-neglect. Seven types of abuse are described: • Physical abuse: inflicting of physical pain or injury, including hitting, slapping, or restraining • Sexual abuse: inflicting of nonconsensual sexual activity of any kind • Psychologic abuse: inflicting mental anguish, including intimidation, humiliation, ridicule, or threats through verbal or nonverbal means • Financial abuse: improper use of the resources of any older person without the elder’s consent for another’s benefit • Abandonment: desertion of an elderly person by the responsible caregiver • Neglect: failure to fulfill a caretaking obligation, including provision of food, a safe living environment, health care, hygiene, or basic custodial care • Self-neglect: behavior of an elderly person that threatens the elder’s health or safety There is considerable variation between states regarding the definitions of abuse and reporting requirements for specific subtypes of abuse.

TREATMENT Section I

DEFINITION

• Neglect is the most common form of elder mistreatment. PREDOMINANT AGE: Risk increases as level of disability increases PEAK INCIDENCE: 80 yr old RISK FACTORS: (Victim) • Poor health • Impaired cognition • Social isolation RISK FACTORS: (Perpetrator) • Substance abuse • Mental illness • Dependence on the victim • Being an involuntary or ill-equipped caregiver • History of violence

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Alcoholism BASIC INFORMATION DEFINITION Although it is impossible to define alcoholism precisely, among the commonly used screening instruments for this disorder are the CAGE questionnaire, short Michigan Alcoholism Screening Test (SMAST), National Council on Alcoholism criteria, and DSM-IV-R criteria. Moderate drinking has been defined as two standard drinks (e.g., 12 oz of beer) per day and one drink per day for women and persons older than 65 years of age. Although not generally included under the alcoholism topic, hazardous or at-risk drinking should also be considered. For men, at-risk drinking is defined as greater than 14 drinks/week or more than 4 drinks/occasion. For women, atrisk drinking is defined as about half that given for men. The American Psychiatric Association defines diagnostic criteria for alcohol withdrawal as follows: A. Cessation of (or reduction in) alcohol use that has been heavy and prolonged. B. Two (or more) of the following, developing within several hours to a few days after criterion A: 1. Autonomic hyperactivity (e.g., sweating or pulse rate 100 beats/ min) 2. Increased hand tremor 3. Insomnia 4. Nausea and vomiting 5. Transient visual, tactile, or auditory hallucinations or illusions 6. Psychomotor agitation 7. Anxiety 8. Grand mal seizures C. The symptoms in criterion B cause clinically significant distress or impairment in social, occupational, or other important areas of functioning. The symptoms are not due to a general medical condition and are not better accounted for by another mental disorder.

PREVALENCE (IN U.S.): 7% of population 18 yr or older PREDOMINANT SEX: • Lifetime risk for males 8% to 10% • Lifetime risk for females 3% to 5% GENETICS: More common with a family history of alcoholism and in patients of Irish, Scandinavian, and Native American descent

PHYSICAL FINDINGS & CLINICAL PRESENTATION • • • • • • • • •

Recurring minor trauma GI bleeding from gastris and/or varices Pancreatitis (acute and chronic) Liver disease Odor of alcohol on breath Tremulousness Tachycardia Peripheral neuropathy Recent memory loss

ETIOLOGY • Social and genetic factors important • Risk factors: 1. Broken homes 2. Unemployment 3. Divorce 4. Recurrent depression 5. Addiction to another substance, including tobacco

DIAGNOSIS WORKUP

SYNONYMS

• Several screening tests (CAGE, AUDIT, SMAST) are available. The four-item CAGE (feeling need to Cut down, Annoyed by criticism, Guilty about drinking, and need for an Eye-opener in the morning) is the most popular screening test in primary care. A positive response should lead to further questioning. The sensitivity of the CAGE ranges from 43% to 94% and its specificity ranges from 70% to 97%. Screening tools are available at the National Institute on Alcohol Abuse and Alcoholism Web site: http://www. niaaa.nih.gov/publications/niaaa-guide. • Blood studies (see “Laboratory Tests”)

Alcohol abuse Substance abuse

LABORATORY TESTS

ICD-9CM CODES 303.9 Alcoholism

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • The clinical history suggests alcohol problems in 15% to 20% of patients in primary care and patients that are hospitalized. In the U.S. alcohol abuse generates nearly $185 billion in annual economic costs. • 20% achieve abstinence without help, 70% achieve sobriety for 1 yr.

• -Glutamyltransferase (GGTP), generally elevated • Liver transaminases (ALT, AST), often elevated, may be normal or low in advanced liver disease • Low albumin level, hypophosphatemia, hypomagnesemia from malnutrition • CBC reveals elevated mean corpuscular volume (MCV) from toxic effect of alcohol on erythrocyte development on nutritional deficiencies • Stool for occult blood may be positive secondary to gastritis, or variceal bleeding

IMAGING STUDIES Indicated only if there is a history of trauma. CT or ultrasound of abdomen may reveal fatty liver or cirrhosis in advanced stages.

TREATMENT NONPHARMACOLOGIC THERAPY • Complete abstinence • Depression, if present, should be treated at same time ETOH is withdrawn

ACUTE GENERAL Rx Alcohol withdrawal syndrome occurs when a person stops ingesting alcohol after prolonged consumption. It can result in four possible clinical patterns depending on the severity of the patient’s alcohol abuse and the time interval from the patient’s previous alcohol ingestion. Blood ethanol level decreases by 20 mg/dL/hr in a normal person. Although discussed separately in the text, these alcohol withdrawal states blend together in real life. 1. Tremulous state: (early alcohol withdrawal, “impending DTs,” “shakes,” “jitters”) a. Time interval: usually occurs 6 to 8 hr after the last drink or 12 to 48 hr after reduction of alcohol intake; becomes most pronounced at 24 to 36 hr b. Manifestation: tremors, mild agitation, insomnia, tachycardia; symptoms are relieved by alcohol c. Inpatient treatment (1) Admit to medical floor (private room); monitor vital signs q4h; institute seizure precautions; maintain adequate sedation. (2) Administer lorazepam as follows: (a) Day 1: 2 mg PO q4h while awake and not lethargic (b) Day 2: 1 mg PO q4h while awake and not lethargic (c) Day 3: 0.5 mg PO q4h while awake and not lethargic (d) NOTE: Hold sedation for lethargy or abnormal vital or neurologic signs. The preceding doses are only guidelines; it is best to titrate the dose case by case (3) In patients with mild to moderate withdrawal and without history of seizures, individualized benzodiazepine administration (rather than a fixeddose regimen) results in lower benzodiazepine administration and avoids unnecessary sedation. The Clinical Institute Withdrawal Assessment-Alco-

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Alcoholism

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peractivity; this is the most serious clinical presentation of alcohol withdrawal (mortality is approximately 15% in untreated patients). c. Treatment (1) Admission to a detoxification unit where patient can be observed closely (2) Vital signs q30min (neurologic signs, if necessary) (3) Use of lateral decubitus or prone position if restraints are necessary (4) NPO: NG tube for abdominal distention may be necessary but should not be routinely used (5) Laboratory studies: same as for early alcohol withdrawal (6) Vigorous hydration (4-6 L/ day): IV with glucose (Na, K, PO43, and Mg2 replacement) (7) Vitamins: thiamine, 100 mg IV qd. The initial dose of thiamine should precede the administration of IV dextrose; multivitamins (may be added to the hydrating solution) (8) Sedation: Control of agitation should be achieved using rapid-acting sedative-hypnotic agents in adequate doses to maintain light somnolence for the duration of delirium. (a) Initially: lorazepam 2 to 5 mg IM/IV repeated prn (b) Maintenance (individualized dosage): chlordiazepoxide, 50 to 100 mg PO q4-6h, lorazepam 2 mg PO q4h, or diazepam 5 to 10 mg PO tid; withhold doses or decrease subsequent doses if signs of oversedation are apparent (c) Midazolam is also effective for managing DTs. Its rapid onset (sedation within 2 to 4 min of IV injection) and short duration of action (approximately 30 min) make it an ideal agent for titration in continuous infusion. (9) Treatment of seizures (as previously described) (10) Diagnosis and treatment of concomitant medical, surgical, or psychiatric conditions

CHRONIC Rx • See “Referral.” • Pharmacotherapies for alcoholism include the opiate antagonists (naltrexone 50 mg PO qd or nalmefene 10 to 40 mg qd), disulfiram, acamprosate, and SSRIs.

DISEASES AND DISORDERS

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(9) Social rehabilitation: group therapy such as Alcoholics Anonymous; identification and treatment of social and family problems should be initiated during the patient’s hospital stay. 2. Alcoholic hallucinosis a. Manifestations: usually hallucinations are auditory, but occasionally hallucinations are visual, tactile, or olfactory; usually there is no clouding of sensorium as in delirium. Disordered perceptions become most pronounced after 24 to 36 hr of abstinence. b. Treatment: same as for DTs (see Withdrawal seizures). 3. Withdrawal seizures (“rum fits”) a. Time interval: usually occurs 7 to 30 hr after cessation of drinking, with a peak incidence between 13 and 24 hr. b. Manifestations: generalized convulsions with loss of consciousness; focal signs are usually absent; consider further investigation with CT scan of head and EEG if clearly indicated (e.g., presence of focal neurologic deficits, prolonged postictal confusion state). In addition, in a febrile patient who is having a seizure or altered mental state, a lumbar puncture is necessary. c. Treatment (1) Diazepam 2.5 mg/min IV until seizure is controlled (check for respiratory depression or hypotension) may be beneficial for prolonged seizure activity; IV lorazepam 1 to 2 mg every 2 hr can be used in place of diazepam. Generally withdrawal seizures are self-limited and treatment is not required; the use of phenytoin or other anticonvulsants for short-term treatment of alcohol withdrawal seizures is not recommended. (2) Thiamine 100 mg IV, followed by IV dextrose, should also be administered. (3) Electrolyte imbalances (↑ Mg2, ↓ K, ↑/↓ Na, ↓ PO43) that may exacerbate seizures should be corrected. 4. DTs: a. Time interval: variable; usually occurs within 1 wk after reduction or cessation of heavy alcohol intake and persists for 1 to 3 days. Peak incidence is 72 hr and 96 hr after the cessation of alcohol consumption. b. Manifestations: profound confusion, tremors, vivid visual and tactile hallucinations, autonomic hy-

Section I

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hol (CIWA-A) scale can be used to measure the severity of alcohol withdrawal. It consists of 10 items: nausea; tremor; autonomic hyperactivity; anxiety; agitation; tactile, visual, and auditory disturbances; headache; and disorientation. Each item is assigned a score from 0 to 7. For example in the “agitation” category 0 indicates normal activity, 7 indicates that the patient constantly thrashes about; for the category of “tremor,” 0 indicates that tremor is not present, 7 tremor is severe, even with arms not extended. The maximum total score is 67. When the CIWA-A score is 8, patients are usually given 2 to 4 mg of lorazepam hourly. -Adrenergic blockers: blockers are useful for controlling BP and tachyarrhythmias. However, they do not prevent progression to more serious symptoms of withdrawal and if used, should not be administered alone but in conjunction with benzodiazepines. Blockers should be avoided in patients with contraindications to their use (e.g., bronchospasm, bradycardia, or severe CHF). Centrally acting adrenergic agonists such as clonidine ameliorate symptoms in patients with mild to moderate withdrawal but do not reduce delirium or seizures. Vitamin replacement: thiamine 100 mg IV or IM for at least 5 days, plus PO multivitamins. The IV administration of glucose can precipitate Wernicke’s encephalopathy in alcoholics with thiamine deficiency; therefore thiamine administration should precede IV dextrose Hydration PO or IV (highcaloric solution): if IV, glucose with Na, K, Mg2, and phosphate replacement prn Laboratory studies (a) CBC, platelet count, INR (b) Electrolytes, glucose, BUN, creatinine (c) GGTP, ALT, AST (d) Phosphorus and magnesium (e) Serum vitamin B12 and folic acid (if megaloblastic features in blood smear) Diagnostic imaging: generally not necessary; if subdural hematoma is suspected (evidence of trauma, persistent lethargy), a CT scan should be ordered.

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Alcoholism DISPOSITION See “Referral.”

REFERRAL • To Alcoholics Anonymous or Adult Children of Alcoholics • Family members to Al-Anon or Al-A-Teen • Many cities have Salvation Army Adult Rehabilitation centers; all patients accepted, regardless of ability to pay

PEARLS & CONSIDERATIONS COMMENTS • Relative indications for inpatient alcohol detoxification are as follows: history of DTs or withdrawal seizures, severe withdrawal symptoms, concomitant psychiatric or medical illness, multiple previous detoxifications, recent high levels of alcohol consumption, and lack of reliable support network. • The cure rate for alcoholism is very disappointing, regardless of the

modality. Only those who want to be helped will be helped. An effective strategy for the primary care physician is a prominently displayed sign in the office that states, “If you think you consume too much alcoholic beverage, please discuss it with me.” Those who do open up the discussion can be given the facts in a nonjudgmental way and often can be helped. All too often, problem drinkers lie on the questionnaire until they face a lifethreatening health issue—and even then denial often reigns supreme.

SUGGESTED READINGS Bayard M et al: Alcohol withdrawal syndrome, Am Fam Physician 69:1443, 2004. Daeppen JB et al: Symptom-triggered vs fixedschedule doses of benzodiazepine for alcohol withdrawal: a randomized treatment trial, Arch Intern Med 162:1117, 2002. Enoch ME, Goldman D: Problem drinking and alcoholism: diagnosis and treatment, Am Fam Physician 65:441, 2002. Fleming MF et al: Brief physician advice for problem drinkers: long-term efficacy and benefit-cost analysis, Alcohol Clin Exp Res 26:36, 2002.

Kosten TR, O’Connor PG: Management of drug and alcohol withdrawal, N Engl J Med 348:1786, 2003. Krystal JH et al: Naltrexone in the treatment of alcohol dependence, N Engl J Med 345:1734, 2001. Mayo-Smith MF et al: Management of alcohol withdrawal delirium, Arch Intern Med 164:1405, 2004. Moyer A et al: Brief interventions for alcohol problems: a meta-analytic review of controlled investigations in treatment-seeking populations, Addiction 97:279, 2002. Nicholas JM et al: The effect of controlled drinking in alcoholic cardiomyopathy, Ann Intern Med 136:192, 2002. O’Connor PG, Schotrenfeld RS: Patients with alcohol problems, N Engl J Med 9:592, 1998. Schneekloth TD et al: Point prevalence of alcoholism in hospitalized patients: continuing challenges of detection, assessment, and diagnosis, Mayo Clin Proc 76:460, 2001. U.S. Preventive Services Task Force: Screening and behavioral counseling interventions in primary care to reduce alcohol misuse: recommendation statement, Ann Intern Med 140:554, 2004. White IR et al: Alcohol consumption and mortality: modelling risks for men and women at different ages, BMJ 325:191, 2002. AUTHOR: FRED F. FERRI, M.D.

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Amyloidosis BASIC INFORMATION

ICD-9CM CODES 277.3 Amyloidosis

EPIDEMIOLOGY & DEMOGRAPHICS • Amyloidosis affects primarily males between the ages of 60 and 70 yr. • There are between 1500 and 3500 new cases annually in the U.S. • The most common type in the U.S. is immunoglobulin light chain related (AL) occurring in 5 to 12 persons/yr in the U.S.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Findings are variable with organ system involvement. Symmetric polyarthritis, peripheral neuropathy, and carpal tunnel syndrome may be present with joint involvement. • Signs and symptoms of nephrotic syndrome may be present with renal involvement. • Fatigue and dyspnea may occur with pulmonary involvement. • Diarrhea, macroglossia (20% of patients), malabsorption, hepatomegaly, and weight loss may occur with GI involvement. • Cardiac involvement is common and can lead to predominantly right-sided CHF, JVD, peripheral edema, and hepatomegaly. • Vascular involvement can result in easy bleeding and periorbital purpura (“raccoon-eyes”).

ETIOLOGY In patients with amyloidosis, a soluble circulating protein (serum amyloid P [SAP]) is deposited in tissues as insoluble -pleated sheets. The source of amyloid protein is a population of monoclonal plasma cells in the bone marrow. There are several chemically documented amy-

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Variable, depending on the organ involvement: • Renal involvement (toxin- or druginduced necrosis, glomerulonephritis, renal vein thrombosis) • Interstitial lung disease (sarcoidosis, connective tissue disease, infectious etiologies) • Restrictive cardiac (endomyocardial fibrosis, viral myocarditis) • Carpal tunnel (rheumatoid arthritis, hypothyroidism, overuse) • Mental status changes (multiinfarct dementia) • Peripheral neuropathy (alcohol abuse, vitamin deficiencies, diabetes mellitus)

WORKUP Diagnostic approach is aimed at demonstration of amyloid deposits in tissues. This may be accomplished with rectal biopsy (positive in 60% of cases). Renal, myocardial, and bone marrow biopsy are other options. Abdominal fat pad biopsy can also be diagnostic; however, its yield is low and it should generally be reserved for evaluation of patients with peripheral neuropathy who also have findings associated with systemic amyloidosis.

LABORATORY TESTS • Initial laboratory evaluation should include CBC, TSH, renal functions studies, ALT, AST, alkaline phosphatase, bilirubin, urinalysis, and serum and urine protein immunoelectrophoresis. • Various laboratory abnormalities include proteinuria (found in 70% of cases), anemia, renal insufficiency, liver function abnormalities, hypothyroidism (10% to 20% of patients), and elevated monoclonal proteins. The finding of a monoclonal light chain in the serum or urine is very useful for diagnosis. • DNA analysis is necessary for the diagnosis of hereditary amyloidosis.

IMAGING STUDIES • Chest x-ray may reveal hilar adenopathy and mediastinal adenopathy. • Two-dimensional Doppler echocardiography to study diagnostic filling is useful to evaluate for cardiac involvement.

TREATMENT ACUTE GENERAL Rx • Therapy is variable, depending on the type of amyloidosis. Amyloidosis associated with plasma cell disorders may be treated with melphalan and prednisone, along with colchicine. Colchicine may also be effective in renal amyloidosis. • Treatment of AL amyloidosis with high-dose melphalan and stem-cell transplantation may result in hematologic remission and improved 5-yr survival. • Promising results have been found with the use of a molecule known as CPHPC given IV or SC in amyloidosis. This molecule has been shown effective in reducing circulating levels of SAP.

CHRONIC Rx Renal transplantation is needed in patients with renal amyloidosis. Peritoneal dialysis in place of hemodialysis in patients with renal failure may improve hemodialysis amyloidosis by clearing -2 microglobulin.

DISPOSITION Prognosis is determined primarily by the presence or absence of cardiac involvement and with the form of amyloidosis: • In reactive amyloidosis, eradication of the predisposing disease slows and can occasionally reverse the progression of amyloid disease. Survival of 5 to 10 yr after diagnosis is not uncommon. • Patients with familial amyloidotic polyneuropathy generally have a prolonged course lasting 10 to 15 yr. • Amyloidosis associated with immunocytic processes carries the worst prognosis (life expectancy 1 yr). • The progression of amyloidosis associated with renal hemodialysis can be improved with newer dialysis membranes that can pass -2 microglobulin. • Median survival in patients with overt CHF is approximately 6 mo, 30 mo without CHF.

SUGGESTED READING Skinner M et al: High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study, Ann Intern Med 140:85, 2004. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Amyloidosis is a generic term describing the deposition of amyloid fibrils in body tissues. Amyloid is an amorphous, eosinophilic material; it is birefringent and usually extracellular. Electron microscopy reveals nonbranching fibrils that are soluble and relatively resistant to proteolytic digestion. There are two major forms of acquired systemic amyloidosis: • AA, associated with chronic inflammatory diseases (e.g., rheumatoid arthritis) and amyloid deposits in kidneys, liver, and spleen • AL (formerly known as primary amyloidosis) affecting the kidneys, heart, liver, intestines, skin, peripheral sensory nervous system, spleen, and lungs

• Nuclear imaging with technetiumlabeled aprotinin may detect cardiac amyloidosis. SAP scintigraphy has high sensitivity for the detection of amyloid deposits in liver, spleen, kidneys, adrenal glands, and bones.

Section I

DEFINITION

loidoses that can be principally subdivided into: 1. Acquired systemic amyloidosis (immunoglobulin light chain, multiple myeloma, hemodialysis amyloidosis) 2. Heredofamilial systemic (polyneuropathy, familial Mediterranean fever) 3. Organ-limited (Alzheimer’s disease) 4. Localized endocrine (pancreatic islet, medullary thyroid carcinoma)

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Amyotrophic Lateral Sclerosis BASIC INFORMATION DEFINITION Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative neuromuscular condition of undetermined etiology affecting corticospinal tracts and anterior horn cells resulting in dysfunction of both upper motor neurons (UMN) and lower motor neurons (LMN), respectively. ICD-9CM CODES 335.20 Amyotrophic lateral sclerosis

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: 0.5 to 2 cases/100,000 persons. Onset is usually between the ages of 50 and 70 years. The male:female ratio is 2:1. PREVALENCE: 5 in 100,000 persons

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Lower motor neuron signs (weakness, hypotonia, wasting, fasciculations, hypoflexia or areflexia) • Upper motor neuron signs (loss of fine motor dexterity, spasticity, extensor plantar responses, hyperreflexia, clonus) • Early deficits may be asymmetric • Preservation of extraocular movements, sensation, bowel and bladder function • Dysarthria, dysphagia, pseudobulbar affect, frontal lobe dysfunction • ALS comprises approximately 90% of adult-onset motor neuron disease. Other presentations of motor neuron disease include progressive muscular atrophy, primary lateral sclerosis, progressive bulbar palsy, progressive pseudobulbar palsy, and ALS-parkinsonism-dementia complex.

ETIOLOGY • 90% to 95% of all cases are sporadic; of the familial cases, approximately 20% are associated with a genetic defect in the copper-zinc superoxide dismutase enzyme (SOD1).

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Multifocal motor neuropathy with conduction block (MMN) • Cervical spondylotic myelopathy with polyradiculopathy • Spinal stenosis with compression of lumbosacral nerve roots • Chronic inflammatory demyelinating polyneuropathy with CNS lesions

• • • • • • • • •

Syringomyelia Syringobulbia Foramen magnum tumor Spinal muscular atrophy (SMA) Late-onset hexosaminidase A deficiency Polyglucosan body disease Bulbospinal muscular atrophy (Kennedy’s disease) Monomyelic amyotrophy ALS-like syndromes have been reported in the setting of lead intoxication, HIV, hyperparathyroidism, hyperthyroidism, lymphoma, and B12 deficiency

WORKUP • EMG and nerve conduction studies • Lumbar puncture to assess protein, serum GM-1 Ab if MMN suspected • Assessment of respiratory function (FVC, NIF)

LABORATORY TESTS • B12, thyroid function, CPK, PTH, HIV may be considered • Serum protein and immunofixation electrophoresis • DNA studies for SMA or bulbospinal atrophy, hexosaminidase levels in pure LMN syndrome • 24-hour urine for lead if indicated

IMAGING STUDIES • Craniospinal neuroimaging contingent upon clinical scenario • Modified barium swallow to evaluate aspiration risk

tients with ALS. Dosage is 50 mg q12h, at least 1 hr before or 2 hr after meals. Shown to prolong survival by 2-3 mo. Manufacturer recommends checking ALT at an initial frequency of once a month for 3 months, followed by once every 3 months until the first year of therapy is completed. ALT should be checked periodically thereafter.

CHRONIC Rx • Glycopyrrolate or amitryptyline to help with sialorrhea (propranolol or metoprolol if secretions are thick in addition to sialorrhea) • Relief of spasticity with baclofen, tizanadine, clonazepam • Treatment of pseudobulbar affect with amitriptyline, sertraline (Zoloft), dextromethorphan

DISPOSITION • Mean duration of symptoms is 3 to 5 yr. • About 20% of patients survive 5 yr.

REFERRAL • Referral to a neurologist experienced in neuromuscular disease is recommended to confirm the diagnosis. • GI referral for PEG placement is recommended while forced vital capacity (FVC) remains 50% to minimize the risks inherent to the procedure.

PEARLS & CONSIDERATIONS

TREATMENT

COMMENTS

NONPHARMACOLOGIC THERAPY

• Patient education material may be obtained through the following: ALS Association, 21021 Ventura Boulevard, Suite 321, Woodland Hills, CA 91364, phone: (800) 782-4727; or the Muscular Dystrophy Association, 3561 East Sunrise Drive, Tucson, AZ 85718-3204, phone: (800) 572-1717, www.mdausa.org/.

• Noninvasive positive pressure ventilation improves quality of life and increases tracheostomy-free survival • PEG placement improves caloric and fluid status, eases medication administration, and may prolong life on the order of 1-4 mo • Nutrition, speech therapy, physical and occupational therapy services • Suction device for sialorrhea • Communication may be eased with computerized assistive devices • Early discussion of living will, recusitation orders, desire for PEG and tracheostomy, potential long-term care options • Encourage contact with local support groups

ACUTE GENERAL Rx Riluzole (Rilutek), a glutamate antagonist, is the only medication approved to extend tracheostomy-free survival in pa-

SUGGESTED READINGS Bradley WG et al: Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology, Neurology 57(3):500, 2001. Miller RG et al: Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review), Muscle Nerve 22(8):1104, 1999. Rowland LP, Shneider NA: Amyotrophic lateral sclerosis, N Engl J Med 344:1688, 2001. AUTHOR: TAYLOR HARRISON, M.D.

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Anemia, Iron Deficiency BASIC INFORMATION Iron deficiency anemia is anemia secondary to inadequate iron supplementation or excessive blood loss.

EPIDEMIOLOGY & DEMOGRAPHICS • Iron deficiency is the most common nutritional deficiency worldwide. • Iron deficiency is never a complete diagnosis. If caused by blood loss, the source of bleeding must be identified.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Most patients have a normal examination. • Skin pallor and conjunctival pallor may be present.

ETIOLOGY • Blood loss from GI (GU blood loss less often the cause) • Dietary iron deficiency (common in patient with dementia, depression, and other causes of failure to thrive) • Poor iron absorption in patients with gastric or small bowel surgery • Repeated phlebotomy • Increased requirements • Other: traumatic hemolysis (abnormally functioning cardiac valves), idiopathic pulmonary hemosiderosis (iron sequestration in pulmonary macrophages), paroxysmal nocturnal hemoglobinuria (intravascular hemolysis)

• Treatment consists of ferrous sulfate 325 mg PO qd for at least 6 mo. Calcium supplements can decrease iron absorption; therefore, these two medications should be staggered. • Parenteral iron therapy is reserved for patients with poor tolerance, noncompliance with oral preparations, or malabsorption. • Transfusion of packed RBCs is indicated in patients with severe symptomatic anemia (e.g., angina) or lifethreatening anemia.

LABORATORY TESTS • Laboratory results vary with the stage of deficiency. • Absent iron marrow stores and decreased serum ferritin are the initial abnormalities. • Decreased serum iron and increased TIBC are the next abnormalities. • Hypochromic microcytic anemia is present with significant iron deficiency. • Peripheral smear in patients with iron deficiency generally reveals microcytic hypochromic RBCs with a wide area of central pallor, anisocytosis, and poikilocytosis when severe. • Laboratory abnormalities consistent with iron deficiency are low serum ferritin level, elevated RBC distribution width (RDW) with values generally 15, low MCV, elevated TIBC, and low serum iron. • The reticulocyte hemoglobin content (CHr) may be a good screening test for iron deficiency. It can be measured on an automated hematology analyzer and represents a relatively inexpensive and fast way to detect iron deficiency.

TREATMENT

DIAGNOSIS

NONPHARMACOLOGIC THERAPY

DIFFERENTIAL DIAGNOSIS

Patients should be instructed to consume foods containing large amounts of iron, such as liver, red meat, and legumes.

• Anemia of chronic disease • Sideroblastic anemia • Thalassemia trait

CHRONIC Rx Patients should be instructed to continue their iron supplements for at least 6 mo or longer to correct depleted body iron stores.

DISPOSITION Most patients respond rapidly to iron supplementation with improvement in CBC and general well-being. GI side effects from oral iron therapy are common and may require decreased dose to once every other day.

REFERRAL GI referral for evaluation of GI malignancy is recommended in all patients with iron deficiency and suspected GI blood loss.

PEARLS & CONSIDERATIONS COMMENTS If the diagnosis of iron deficiency anemia is made, it is mandatory to try to locate the suspected site of iron loss.

SUGGESTED READING Tefferi A: Anemia in adults: a contemporary approach to diagnosis, Mayo Clin Proc 78:1274, 2004. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

ICD-9CM CODES 280.9 Iron deficiency anemia

ACUTE GENERAL Rx

Diagnostic workup consists primarily of laboratory evaluation. Most patients with iron deficiency anemia are asymptomatic in the early stages. With progressive anemia, the major complaints are fatigue, dizziness, exertional dyspnea, pagophagia (ice eating), and pica. Patient’s history may also suggest GI blood loss (melena, hematochezia, hemoptysis).

Section I

DEFINITION

WORKUP

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Anemia, Pernicious

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BASIC INFORMATION DEFINITION Pernicious anemia is an autoimmune disease resulting from antibodies against intrinsic factor and gastric parietal cells.

SYNONYMS Megaloblastic anemia resulting from vitamin B12 deficiency ICD-9CM CODES 281.0 Pernicious anemia

EPIDEMIOLOGY & DEMOGRAPHICS • Increased incidence in females and older adults • The overall prevalence of undiagnosed PA over age 60 yr is 1.9% • Prevalence is highest in women (2.7%), particularly in black women (4.3%) • Increased incidence of autoimmune disease (e.g., type 1 DM, Graves’ disease, Addison’s disease), Helicobacter pylori infection

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Mucosal pallor, glossitis • Peripheral sensory neuropathy with paresthesias initially and absent reflexes in advanced cases • Loss of joint position sense, pyramidal or long track signs • Possible splenomegaly and mild hepatomegaly • Generalized weakness and delirium/ dementia

ETIOLOGY • Antigastric parietal cell antibodies in 70% of patients, antiintrinsic factor antibodies in 50% of patients • Atrophic gastric mucosa

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Nutritional vitamin B12 deficiency Malabsorption Chronic alcoholism (multifactorial) Chronic gastritis related to H. pylori infection • Folic acid deficiency • Myelodysplasia

WORKUP • The clinical presentation of pernicious anemia varies with the stage. Initially, patients may be asymptomatic. In advanced stages, patients may present with impaired memory, depression,

gait disturbances, paresthesias, and complaints of generalized weakness. • Investigation consists primarily of laboratory evaluation. • Endoscopy and biopsy for atrophic gastritis may be performed in selected cases. • Diagnosis is crucial because failure to treat may result in irreversible neurologic deficits.

LABORATORY TESTS • CBC generally reveals macrocytic anemia and leukopenia with hypersegmented neutrophils. • MCV is generally significantly elevated in the advanced stages. • Reticulocyte count is low/normal. • Falsely low serum cobalamin levels can occur in patients with severe folate deficiency, in patients using high doses of ascorbic acid, and when cobalamin levels are measured following nuclear medicine studies (radioactivity interferes with cobalamin RIA measurement). • Falsely high normal levels in patients with cobalamin deficiency can occur in severe liver disease or chronic granulocytic leukemia. • The absence of anemia or macrocytosis does not exclude the diagnosis of cobalamin deficiency. Anemia is absent in 20% of patients with cobalamin deficiency, and macrocytosis is absent in 30% of patients at the time of diagnosis. It can be blocked by concurrent iron deficiency or anemia of chronic disease and may be masked by thalassemia trait. • Schilling test is abnormal in part I; part II corrects to normal after administration of intrinsic factor. • Laboratory tests used for detecting cobalamin deficiency in patients with normal vitamin B12 levels include serum and urinary methylmalonic acid level (elevated), total homocysteine level (elevated), intrinsic factor antibody (positive). • An increased concentration of plasma methylmalonic acid (P-MMA) does not predict clinical manifestations of vitamin B12 deficiency and should not be used as the only marker for diagnosis of B12 deficiency. • Additional laboratory abnormalities can include elevated LDH, direct hyperbilirubinemia, and decreased haptoglobin. • Antiparietal antibody test should be positive in pernicious anemia and negative in nutritional vitamin B12 deficiency.

TREATMENT NONPHARMACOLOGIC THERAPY Avoid folic acid supplementation without proper vitamin B12 supplementation.

ACUTE GENERAL Rx • Traditional therapy of a cobalamin deficiency consists of IM injections of vitamin B12 1000 g/wk for the initial 4 to 6 wk followed by 1000 g/mo IM indefinitely. When hematologic parameters have returned to normal range, intranasal cyanocobalamin may be used in place of IM cyanocobalamin. The initial dose of intranasal cyanocobalamin (Nascobal) is one spray (500 g) in one nostril once per week. Monitor response and increase dose if serum B12 levels decline. Consider return to intramuscular vitamin B12 supplementation if decline persists. • Oral vitamin B12 replacement is appropriate (1 mg/day) for nutritional vitamin B12 deficiency.

CHRONIC Rx Parenteral vitamin B12 1000 g/mo or intranasal cyanocobalamin 500 g/wk (see “Acute General Rx”) for the remainder of life

DISPOSITION Anemia generally resolves with appropriate treatment. Neurologic deficits, if present at diagnosis, may be permanent.

REFERRAL Consider GI referral for endoscopy upon diagnosis of pernicious anemia and surveillance endoscopy every 5 yr to rule out gastric carcinoma

PEARLS & CONSIDERATIONS COMMENTS • Patients must understand that therapy is lifelong. • Self-injection of vitamin B12 may be taught in selected patients. • Oral cobalamin (1000 /day) has been reported as also being effective in mild cases of pernicious anemia because about 1% of an oral dose is absorbed by passive diffusion, a pathway that does not require intrinsic factor. AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

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Aneurysm, Abdominal Aorta BASIC INFORMATION

DIAGNOSIS WORKUP • Abdominal ultrasound is nearly 100%

from 12.2 cases/100,000 persons to 36.2 cases/100,000 persons from 1951 to 1980. • The prevalence ranges from 2% to 5% in men 60 yr. • AAA is predominantly a disease of the elderly, affecting men women (4:1). • Rupture of an AAA is the tenth leading cause of death in men 55 yr (15,000 deaths/yr in the U.S.).

accurate in identifying an aneurysm and estimating the size to within 0.3 to 0.4 cm. It is not very good in estimating the proximal extension to the renal arteries or involvement of the iliac arteries. • CT scan is recommended for preoperative aneurysm imaging and estimating the size to within 0.3 mm. There are no false-negatives, and the CT scan can localize the proximal extent, detect the integrity of the wall, and rule out rupture. • Angiography gives detailed arterial anatomy, localizing the aneurysm relative to the renal and visceral arteries. This is the definitive preoperative study for surgeons. • MRI can also be used, but it is more expensive and not as readily available.

PHYSICAL FINDINGS & CLINICAL PRESENTATION

TREATMENT

SYMPTOMS • Abdominal pain radiating to the back, flank, and groin. The pain is thought to be caused by rapid expansion of the aneurysm as it stretches the overlying peritoneum. AAA should be considered in the differential of anyone presenting with abdominal pain or back pain. • Early satiety, nausea, and vomiting due to compression of adjacent bowel. PHYSICAL EXAM • The exam, although not very sensitive for AAA 5 cm in size, has a sensitivity of 82% for detecting AAA 5 cm. • Pulsatile epigastric mass that may or may not be tender. • Venous thrombosis from iliocaval venous compression. • Discoloration and pain of the feet with distal embolization of the thrombus within the aneurysm. • Shock, hypoperfusion, abdominal distention if rupture occurs. Rare presentations include hematemesis or melena with abdominal and back pain in patients with aortoenteric fistulas. Aortocaval fistula produces loud abdominal bruits.

Treatment focuses on risk factor modification (diet and exercise for blood pressure, cholesterol, and diabetes, and abstinence from tobacco) and surgery. Serial studies have shown that expansion rates are faster in current smokers than ex-smokers. • On diagnosing an AAA, surveillance ultrasound for sizing is safe with very low rates of AAA rupture ( 1%) with recommendations for prophylactic surgery for AAA 5.5 cm. • The most commonly used predictor of rupture is the maximum diameter of the AAA. For AAAs with baseline diameters 3.5 cm, 4.0 cm, 4.5 cm, and 5 cm, the recommending screening intervals can be 36, 24, 12, and 3 months, respectively. • Recent randomized trials found no reduction in mortality from repairing AAAs smaller than 5.5 cm in patients at low operative risk. • For aneurysms 5.5 cm or greater, prosthetic graft replacement is recommended, providing there is no contraindication (e.g., MI within 6 mo, refractory CHF, life expectancy 2 yr, severe residual from CVA). • For the high-risk patient deemed inoperable for such major surgery, endovascular stent-anchored grafts under local anesthesia have provided an alternative approach.

ICD-9CM CODES 441.3 Ruptured abdominal aortic aneurysm 441.4 Aneurysm, abdominal (aorta)

EPIDEMIOLOGY & DEMOGRAPHICS • The incidence of AAAs has been rising

ETIOLOGY • Atherosclerotic (degenerative or nonspecific) • Trauma • Cystic medial necrosis (Marfan’s syndrome)

SUGGESTED READINGS Lederle FA: Ultrasonographic screening for abdominal aortic aneurysm, Ann Intern Med 139:516, 2003. Lederle FA et al: Immediate repair compared with surveillance of small abdominal aortic aneurysms, N Engl J Med 346:1437, 2002. Lederle FA et al: Rupture rate of large abdominal aortic aneurysms in patients refusing or unfit for elective repair, JAMA 287:2968, 2002. Powell J, Brady A: Detection, management and prospects for medical treatment of small abdominal aortic aneurysms, Arteroscler Thromb Vasc Biol 24:241, 2004. Powell J, Greenhalgh R: Small abdominal aortic aneurysms, N Engl J Med 348:1895, 2003. The Propranolol Aneurysm Trial Investigators: Propranolol for small abdominal aortic aneurysms: results of a randomized trial, J Vasc Surg 35:72, 2002. Sparks AR et al: Imaging of abdominal aortic aneurysms, Am Fam Physician 65:1565, 2002. The United Kingdom Small Aneurysm Trial Participants: Long-term outcomes of immediate repair compared with surveillance of small abdominal aortic aneurysms, N Engl J Med 346:1445, 2002. AUTHORS: PRANAV M. PATEL, M.D., WEN-CHIH WU, M.D., and TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

An abdominal aortic aneurysm (AAA) is a permanent localized dilation of the abdominal aortic artery to at least 50% when compared with the normal diameter. The normal diameter in men is 2.3 cm, and in women it is 1.9 cm.

• Abdominal aortic rupture is an emergency. Surgery is the only chance for survival. • Most AAAs are infrarenal. Surgical risk is increased in patients with coexisting coronary artery disease, pulmonary disease (Pao2 50 mm Hg, FEV1 11), liver cirrhosis, and chronic renal failure (Cr 3 mg/dl). Cardiac preoperative evaluation with radionuclide perfusion studies for ischemia and aggressive perioperative hemodynamic monitoring help identify highrisk patients and decrease postoperative complications. • In patients with dementia, postoperative delirium should be anticipated and managed. • It is estimated that AAAs 5 cm expand at a rate of 0.4 cm/yr. • The risk of rupture is 0% per year in aneurysms 4 cm, 0.6%-1%/yr in aneurysms 4.0 to 5.5 cm, 4.4%/yr in aneurysms 5.5 to 5.9 cm, 10.2%/yr in aneurysms 6.0 to 6.9 cm, and 32.5%/yr in aneurysms 7 cm. • Mortality after rupture is 90%. Of those patients who reach the hospital, it is estimated 50% will survive compared with a 4% mortality rate for elective repair of the nonruptured aorta. • The use of the (-blocker propranolol has demonstrated a trend toward fewer surgeries in patients with asymptomatic small AAAs (3.0-5.0 cm).

Section I

DEFINITION

• Arteritis, inflammatory • Mycotic, infected (septic) • Syphilis

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Angina Pectoris BASIC INFORMATION DEFINITION Angina pectoris is characterized by discomfort that occurs when myocardial oxygen demand exceeds the supply. Myocardial ischemia can be asymptomatic (silent ischemia), particularly in diabetics. Angina can be classified as follows: 1. CHRONIC (STABLE): • Usually follows a precipitating event (e.g., climbing stairs, sexual intercourse, a heavy meal, emotional stress, cold weather) • Generally same severity as previous attacks; relieved by the customary dose of nitroglycerin • Caused by a fixed coronary artery obstruction secondary to atherosclerosis 2. UNSTABLE (REST OR CRESCENDO, CORONARY SYNDROME): • Recent onset • Increasing severity, duration, or frequency of chronic angina • Occurs at rest or with minimal exertion

FUNCTIONAL CLASSIFICATION • New York Heart Association Functional Classification of Angina: Class I—Angina only with unusually strenuous activity. Class II—Angina with slightly more prolonged or rigorous activity than usual. Class III—Angina with usual daily activity. Class IV—Angina at rest. • Grading of Angina by the Canadian Cardiovascular Society Classification System: Class I—Ordinary physical activity does not cause angina, such as walking, climbing stairs. Angina (occurs) with strenuous, rapid, or prolonged exertion at work or recreation. Class II—Slight limitation of ordinary activity. Angina occurs on walking or climbing stairs rapidly; walking uphill; walking or stair climbing after meals, in cold, in wind, or under emotional stress; or only during the few hours after awakening. Angina occurs on walking more than two blocks on the level and climbing more than one flight of ordinary stairs at a normal pace and in normal condition. Class III—Marked limitations of ordinary physical activity. Angina occurs on walking one to two blocks on the level and climbing one flight of stairs in normal conditions and at a normal pace. Class IV—Inability to carry on any physical activity without discom-

fort—anginal symptoms may be present at rest. ICD-9CM CODES 411.1 Angina, stable 413 Angina pectoris 413.1 Prinzmetal’s angina 413.9 Angina, unspecified

EPIDEMIOLOGY & DEMOGRAPHICS • Angina is most common in middleaged and elderly males. • Females are usually affected after menopause. • Within 12 mo of initial diagnosis, 10% to 20% of patients with diagnosis of stable angina progress to MI or unstable angina.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Although there is significant individual variation, most patients complain of substernal chest pain (pressure, tightness, heaviness, sharp pain, sensation similar to intestinal gas or dysphagia). • The pain is of short duration (30 sec to 30 min), nonpleuritic, and often accompanied by shortness of breath, nausea, diaphoresis, and numbness or pain in the left arm, jaw, or shoulder.

ETIOLOGY UNCONTROLLABLE RISK FACTORS FOR ANGINA: • Advanced age • Male sex • Genetic predisposition MODIFIABLE RISK FACTORS FOR ANGINA: • Smoking (risk is almost double) • Hypertension (risk is double if systolic blood pressure is 180 mm Hg) • Hyperlipidemia • Impaired glucose tolerance or diabetes mellitus • Obesity (weight 30% over ideal) • Hypothyroidism • Left ventricular hypertrophy (LVH) • Sedentary lifestyle • Low serum folate levels (Folate is required for conversion of homocysteine to methionine. Folate deficiencies are associated with an increased risk of fatal coronary heart disease.) • Elevated homocysteine levels. Elevated plasma homocysteine level is a strong and independent risk factor for CHD events especially in patients with type 2 DM • Elevated levels of highly sensitive Creactive protein (hs-CRP, cardio CRP) • Elevated levels of lipoprotein-associated phospholipase A2 • Elevated fibrinogen levels • Depression • Vasculitis

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Noncardiac pain mimicking angina may be caused by: • Pulmonary diseases (pulmonary hypertension, pulmonary embolism, pleurisy, pneumothorax, pneumonia) • GI disorders (peptic ulcer disease, pancreatitis, esophageal spasm or spontaneous esophageal muscle contraction, esophageal reflux, cholecystitis, cholelithiasis) • Musculoskeletal conditions (costochondritis, chest wall trauma, cervical arthritis with radiculopathy, muscle strain, myositis) • Acute aortic dissection • Herpes zoster • Anxiety disorder

WORKUP • In patients presenting with chest pain, the probability of CAD should be estimated on the basis of patient age, sex, cardiovascular risk factors, and pain characteristics. • The most important diagnostic factor is the history. Chest pain or left arm pain or discomfort reproducing previously documented angina and a known history of CAD or MI are indicative of high likelihood of actue coronary syndrome. • The physical examination is of little diagnostic help and may be totally normal in many patients, although the presence of an S4 gallop is suggestive of ischemic chest pain. Transient mitral regurgitation, hypotension, diaphoresis, and rales indicate a high likelihood of acute coronary syndrome. • An ECG taken during the acute episode may show transient T-wave inversion or ST-segment depression or elevation, but more than 50% of patients with chronic stable angina have normal results on resting ECG. • Patients with intermediate or high probability should undergo risk stratification through further testing. Treadmill exercise tolerance test is useful to identify patients with coronary artery disease who would benefit from cardiac catheterization. Stress echocardiogram or radionuclide testing (e.g., thallium, Persantine, dobutamine) are useful and sensitive in the detection of myocardial ischemia. • Although invasive, coronary angiography remains the gold standard for the identification of clinically significant coronary artery disease. Coronary magnetic resonance angiography can also detect coronary artery disease of the proximal and middle segments. This noninvasive approach, where available, can be used to reliably iden-

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Angina Pectoris

LABORATORY TESTS

IMAGING STUDIES • Echocardiography is indicated in patients with systolic murmur suggestive of aortic stenosis, mitral valve prolapse, or hypertrophic cardiomyopathy. It is also useful in the detection of ischemia-induced regional wall motion abnormalities or mitral regurgitation. Echocardiography combined with treadmill exercise (stress echo) or pharmacologic stress with dobutamine can be used to detect regional wall abnormalities that occur during myocardial ischemia associated with CAD. • Coronary angiography is performed to define the location and extent of coronary disease; this is indicated in selected patients who are candidates for CABG surgery or angioplasty. • Noninvasive methods for assessing myocardial viability to predict which patients will have increased LVEF and improved survival after revascularization include positron-emission tomography, dobutamine echocardiography, and contrast-enhanced MRI. Additional studies are needed to determine the

TREATMENT NONPHARMACOLOGIC THERAPY • Aggressive modification of preventable risk factors (weight reduction in obese patients, regular aerobic exercise program, correction of folate deficiency, low-cholesterol and lowsodium diet, cessation of tobacco use) • Diets using nonhydrogenated unsaturated fats as the predominant form of dietary fat, whole grains as the main form of carbohydrates, an abundance of fruits and vegetables, and adequate omega-3-fatty acids are optimal for prevention of coronary heart disease • Correction of possible aggravating factors (e.g., anemia, hypertension, diabetes mellitus, hyperlipidemia, thyrotoxicosis, hypothyroidism)

ACUTE GENERAL Rx The major classes of antiischemic agents are nitrates, -adrenergic blockers, calcium channel blockers, aspirin, and heparin; they can be used alone or in combination. • Nitrates cause venodilation and relaxation of vascular smooth muscle; the decreased venous return from venodilation decreases diastolic ventricular wall tension (preload) and thereby reduces mechanical activity (and myocardial oxygen consumption) during systole. Relaxation of vascular smooth muscle increases coronary blood flow and reduces systemic pressure. Tolerance to nitrates can be minimized by avoiding sustained blood levels with a daily nitrate-free period (e.g., omission of bedtime dose of oral isosorbide dinitrate or 12 hr on/12 hr off transdermal nitroglycerin therapy). Nitrates are relatively contraindicated in patients with hypertrophic obstructive cardiomyopathy, and should also be avoided in patients with severe aortic stenosis. • -Adrenergic blockers achieve their major antianginal effect by reducing heart rate and systolic blood pressure. Absent contraindications, they should be regarded as initial therapy for stable angina for all patients. Their dose should generally be adjusted to reduce the resting heart rate to 50-60 beats/min. • Long-acting calcium channel blockers are usually used as second-line agents. Short-acting calcium channel blockers should be avoided. Calcium channel blockers should generally also be avoided after complicated MI (CHF)

and in patients with CHF secondary to systolic dysfunction (unless necessary to control heart rate). • Aspirin: give initial dose of at least 160 mg/day followed by 81 to 325 mg/day. Aspirin inhibits cyclooxygenics and synthesis of thromboxane A2 and reduces the risk of adverse cardiovascular events by 33% in patients with unstable angina. Patients intolerant to aspirin can be treated with the antiplatelet agent clopidogrel. Clopidogrel acts by irreversibly blocking the P2Y12 adenosine diphosphate receptor on the platelet surface, thereby interrupting platelet activation and aggregation. • Heparin is useful in patients with unstable angina and reduces the frequency of MI and refractory angina. Patients with unstable angina treated with aspirin plus heparin have a 32% reduction in the risk of MI and death compared with those treated with aspirin alone; therefore, unless heparin is contraindicated, most hospitalized patients with unstable angina should be treated with both aspirin and heparin. Enoxaparin (low–molecular weight heparin) 1 mg bid SC is as effective as continuous unfractionated heparin in reducing the incidence of unstable angina. It is usually given for 3-8 days, or until coronary revascularization is performed. Longer administration does not provide additional cardiac benefits and may increase risk of hemorrhage. • Early administration of platelet glycoprotein IIb/IIIa receptor antagonists is useful in addition to aspirin and heparin in patients with unstable angina, in high-risk patients with positive troponin tests, or those undergoing percutaneous revascularization. Abciximab, the first GP IIb/IIa inhibitor, is an important component of percutaneous revascularization. Started in the catheterization lab, it reduces the incidence of ischemic events. Abciximab is contraindicated in patients for whom an early invasive strategy is not planned. Contraindications to the use of GP IIb/IIa inhibitors are: severe hypertension (180/110), internal bleeding within 30 days, history of intracranial hemorrhage, neoplasm, NVM, aneurysm, CVA within 30 days or history of hemorrhagic CVA, thrombocytopenia ( 100 k), acute pericarditis, history or symptoms suggestive of aortic dissection, and major surgical procedures or severe physical trauma within previous month.

CHRONIC Rx Use of lipid-lowering drugs is recommended in patients with coronary heart

DISEASES AND DISORDERS

• Initial laboratory tests in patients with chronic stable angina should include hemoglobin, fasting glucose, and fasting lipid panel. • Cardiac isoenzymes (CK-MB q8h  2) should be obtained to rule out MI in patients presenting with acute chest pain. • Cardiac troponin I and T are specific markers of myocardial necrosis and are useful in evaluating patients with acute chest pain. Elevation of either of these proteins in the setting of an acute coronary syndrome identifies patients with a several-fold increased risk of death in subsequent weeks. Patients with negative troponin assays on arrival in the ER and repeated 4 hr later are at a low level of risk for cardiac events within the following 30 days, and most of these patients can be safely discharged from the ER. Troponin T tests can be false-positive in patients with renal failure, sepsis, rhabdomyolysis, fibrin clots, and heterophile antibodies. The presence of jaundice or the concurrent use of heparin can result in underestimation of troponin. • Cardio-CRP (hs-CRP)—elevation of cardio-CRP is a relatively moderate predictor of coronary heart disease and it adds prognostic information to that conveyed by the Framingham risk score. However, based on current data, it may be premature to adapt widespread assessment of cardio-CRP.

cost effectiveness of these studies in patients with ischemic cardiomyopathy.

Section I

tify (or rule out) left main coronary artery or three-vessel disease.

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Angina Pectoris disease and in patients with hyperlipidemia refractory to diet and exercise. Among patients who have recently had an acute coronary syndrome, an intensive lipid-lowering statin regimen to reduce LDL cholesterol to 70 mg/dl provides greater protection against death or major cardiovascular events than does a standard regimen. Statins also decrease the level of the inflammatory marker hsCRP independently of the magnitude of change in lipid parameters.

REFERRAL Surgical therapy: CABG surgery is recommended for patients with left main coronary disease, for those with symptomatic three-vessel disease, and for those with left ventricular EF 40% and critical (70% stenosis) in all three major coronary arteries. Surgical therapy improves prognosis, particularly in diabetic patients with multivessel disease. Minimally invasive direct coronary artery bypass (MIDCAB) is a variation of CABG for patients in whom sternotomy and cardiopulmonary bypass is either contraindicated or unnecessary. In this procedure the left internal mammary is anastomosed to the LAD through a thoracic incision without cardiopulmonary bypass. This operation is generally performed for patients with only single-vessel CAD. The Port-Access Procedure is another type of minimally invasive technique. Angioplasty and coronary stents: Percutaneous coronary intervention (PCI) should be considered for patients with one- or two-vessel disease that does not involve the main left coronary artery and in whom ventricular function is normal or near normal. Patients selected for PCI should also be candidates for CABG. The types of lesions best suited for angioplasty are proximal lesions, noncalcified, concentric, and preferably shorter than 5 mm (should not exceed 10 mm). Approximately 80% of patients show immediate benefit after PCI. The frequency of abrupt closure postangioplasty can be reduced by pretreatment with IV glycoprotein IIb/IIIa receptor inhibitors, which block the final common pathway of platelet aggregation. In patients with clinically documented acute coronary syndrome who are treated with GP IIb/IIa inhibitors, even small elevations in cTmI and cTmT identify high-risk patients who derive a large clinical benefit from an early invasive strategy. Abciximab (ReoPro) and eptifibatide (Integrilin) are approved for use before and during percutaneous coronary interventions. They are expensive ($1400 per

dose of abciximab) and can cause thrombocytopenia in 0.5% to 1% of patients. Platelet counts should be monitored for 24 hours after starting glycoprotein IIb/IIIa inhibitors. Reversal of thrombocytopenia (e.g., patients undergoing emergency CABG) can be achieved with platelet transfusions. The development of coronary stents has broadened the number of patients who can be treated in the cardiac laboratory. Cardiac stents are currently used in nearly 95% of all percutaneous interventional lesions. The rate of restenosis may be reduced by placing a stent electively in primary atheromatous lesions. In patients with symptomatic isolated stenosis of the proximal left anterior descending artery, stenting has advantages over standard coronary angioplasty in that it is associated with both a lower rate of restenosis and a better clinical outcome. The major limitations of stenting are subacute thrombosis, restenosis within the stent, bleeding complications when anticoagulants are used post-stenting, and higher cost ($1500 average unit price). The combination of aspirin and clopidogrel is effective in preventing coronary stent thrombosis. Vitamin therapy to lower homocysteine levels has been recommended by some for the prevention of restenosis after coronary angioplasty; however, recent reports indicate that the administration of folate, vitamin B6, and vitamin B12 after coronary stenting may increase the risk of instent restenosis and the need for targetvessel revascularization. Stents coated with sirolimus have been shown to dramatically reduce the incidence of stent restenosis by inhibiting the growth of endothelium and fibrosis within the lumen of the stent on the short term. Stents coated with paclitaxel, which inhibits cellular replication and reduces proliferation and migration of endothelial smooth muscle cells, are also effective in reducing the incidence of restenosis. CO2 laser revascularization: This operation is performed only in selected centers and consists of placing 1mm laser channels in the heart muscle. It may be indicated in selected Class III or Class IV angina patients who are failing maximum medical therapy and are not amenable to any other PTCA or coronary bypass surgery.

PEARLS & CONSIDERATIONS COMMENTS Although nitrate responsiveness is usually an integral part of a diagnostic strat-

egy for chronic stable chest pain, recent reports question its value and conclude that in a general population admitted for chest pain, relief of pain after nitroglycerin treatment does not predict active coronary artery disease and should not be used to guide diagnosis in the acute care setting.

SUGGESTED READINGS Aviles RJ et al: Troponin T levels in patients with acute coronary syndromes, with or without renal dysfunction, N Engl J Med 346:2047, 2002. Brennan ML et al: Prognostic value of myeloperoxidase in patients with chest pain, N Engl J Med 349:1595, 2003. Buffon A et al: Widespread coronary inflammation in unstable angina, N Engl J Med 347:5, 2002. Henrikson C et al: Chest pain relief by nitroglycerin does not predict active coronary artery disease, Ann Intern Med 139:979, 2003. Hu F, Willet W: Optimal diets for prevention of coronary heart disease, JAMA 288:2569, 2002. Kushner I, Sehgal A: Is high-sensitivity C-Reactive protein an effective screening test for cardiovascular risk? Arch Intern Med 162:867, 2002. Lange H et al: Folate therapy and in-stent restenosis after coronary stenting, N Engl J Med 350:2673, 2004. Levinson SS, Elin RJ: What is C-reactive protein telling us about coronary artery disease, Arch Intern Med 162:389, 2002. Moses JW et al: Sirolimus-eluting stents versus standard stents in patients with stenosis in a native coronary artery, N Engl J Med 349:1315, 2003. Ridker PM et al: Plasma homocysteine concentration, statin therapy, and the risk of first acute coronary events, Circulation 105:1776, 2002. Ridker PM et al: Comparison of C-reactive protein and low-density lipoprotein cholesterol levels in the prediction of first cardiovascular events, N Engl J Med 347:1557, 2002. Serruys PW et al: Fluvastatin for prevention of cardiac events following successful first percutaneous coronary intervention, JAMA 287:3215, 2002. Snow V et al: Evaluation of primary care patients with chronic stable angina: Guidelines from the American College of Physicians, Ann Intern Med 141:57 and 562, 2004. Soinio M et al: Elevated plasma homocysteine level is an independent predictor of coronary artery disease events in patients with type 2 DM, Ann Intern Med 140:94, 2004. Yang EHC et al: Current and future treatment strategies for refractory angina, Mayo Clin Proc 79(10):1284, 2004. AUTHOR: FRED F. FERRI, M.D.

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Anxiety (Generalized Anxiety Disorder) BASIC INFORMATION

SYNONYMS Anxiety neurosis Chronic anxiety GAD ICD-9CM CODES F41.1 (DSM-IV Code 300.02)

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 31% in 1 yr PREVALENCE (IN U.S.): • In general population: prevalence of 5% lifetime • In primary care setting: 3% (It is the most common anxiety disorder in this setting.) PREDOMINANT SEX: Females are more frequently affected (2:1 ratio), but they present for treatment less frequently (3:2 female:male). PEAK INCIDENCE: Chronic condition with onset in early life GENETICS: Concordance rates in dizygotic twins and monozygotic twins are not different (0% to 5%), but detailed analysis of 1033 female twin pairs finds that heredity contributes about 30% of the factors that may cause GAD.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Report of being “anxious” all of their lives • Excessive worry, usually regarding family, finances, work, or health • Sleep disturbance, particularly early insomnia • Muscle tension (typically in the muscles of neck and shoulders) • Headaches (muscle tension) • Difficulty concentrating • Day form of fatigue • Gastrointestinal symptoms compatible with IBD (one third of patients) • Physical consequences of anxiety are the driving force for patients seeking medical attention

ETIOLOGY • There is no clear etiology. • Several hypotheses centering on neurotransmitter (catecholamines, indolamines) and developmental psychology are used as framework for treatment recommendations. • Risk factors include a family history, increase in stress, history of physical or emotional trauma, and medical illness.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Wide range of psychiatric and medical conditions; however, for a diagnosis of GAD to be made a person must experience anxiety with coexisting physical symptoms the majority of the time continuously for at least 6 mo • Cardiovascular and pulmonary disease • Hyperthyroidism • Parkinson’s disease • Consequence of recreational drug use (e.g., cocaine, amphetamine, and PCP) or withdrawal (e.g., alcohol or benzodiazepines)

WORKUP • History: required for diagnosis • Physical examination: confirm the patient’s physical complaints • Exclusion of organic basis for the complaints possibly requiring additional workup • Physical cause should be suspected if anxiety follows recent changes in medication

TREATMENT NONPHARMACOLOGIC THERAPY • • • •

Cognitive-behavioral therapy Relaxation training Biofeedback Psychodynamic psychotherapy NOTE: Studies directly comparing medications with psychotherapy are not available, but the general clinical impression is that the psychotherapies are probably superior to pharmacotherapies.

ACUTE GENERAL Rx • Acute treatment is rarely indicated because GAD is a chronic condition. • Occasionally, patients are in acute distress, requiring physician to respond quickly; benzodiazepines are given under these conditions as drug of choice for both daytime anxiety and initial insomnia.

Caution should be taken in prescribing benzodiazepines because of the propensity for misuse and dependence in this population. If provided, the patient should be educated about the use of other medications and the risks in using benzodiazepines.

CHRONIC Rx • SSRIs and venlafaxine are also effective in generalized anxiety disorders and are typically given as a first line treatment. These are particularly useful if comorbid depression is present. • If used and prescribed with supervision, benzodiazepines can provide long-term symptom control with only occasional problems with tolerance or abuse; however, tolerance to benzodiazepines is common and for this reason they have fallen from a first line treatment to second line treatment for GAD. Further, the rate of relapse after discontinuation of benzodiazepines may be twice the rate after discontinuation of the available nonbenzodiazepine anxiolytic buspirone. Finally, benzodiazepines can contribute to cognitive impairment in elderly patients, with or without dementia. • Buspirone is effective without any potential for tolerance or abuse. • Sedating antidepressants are also useful in ameliorating initial insomnia.

DISPOSITION • This condition is chronic with periodic exacerbations. • Treatment is given to provide a significant degree of improvement, but symptoms and dysfunction may persist. • The risk for suicide is higher than general population.

REFERRAL • If the symptoms are refractory to treatment • If the case is complicated with a comorbid psychiatric condition • If treatment response is suboptimal with residual dysfunction

SUGGESTED READINGS Fricchione G: Clinical practice. Generalized anxiety disorder, N Engl J Med 351(7):675, 2004. Goodman WK: Selecting pharmacotherapy for generalized anxiety disorder, J Clin Psychiatry 65(suppl 13):8, 2004. Lang AJ: Treating generalized anxiety disorder with cognitive-behavioral therapy, J Clin Psychiatry 65(suppl 13):14, 2004. Rouillon F: Long term therapy of generalized anxiety disorder, Eur Psychiatry 19(2):96, 2004. AUTHORS: PATRICIA AREAN, PH.D., and MITCHELL D. FELDMAN, M.D., M.PHIL.

DISEASES AND DISORDERS

GAD is most likely to present in combination with other psychiatric and medical conditions. GAD commonly presents with excessive anxiety, fear, and worry for most of the time, continuously for at least 6 mo. The subjective anxiety must be accompanied by at least three somatic symptoms (e.g., restlessness, irritability, sleep disturbance, muscle tension, difficulty concentrating, or fatigability). Because worry is also a symptom of depression, officially, GAD is not present if there is a concurrent Major Depression. However, the field is recognizing that the two conditions can coexist, and research on the commonalities are being conducted.

NOTE:

Section I

DEFINITION

• Comorbid psychiatric illness (e.g., dysthymia or major depression) and substance abuse (e.g., alcohol abuse) are frequent

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Aortic Dissection BASIC INFORMATION DEFINITION Aortic dissection occurs when an intimal tear allows blood to dissect between medial layers of the aorta. ICD-9CM CODES 441.00 Aortic dissection 444.01 Aortic dissection, thoracic

SYNONYMS Dissecting aortic aneurysm, unspecified site

EPIDEMIOLOGY & DEMOGRAPHICS PREDOMINANT SEX: Males  females PEAK INCIDENCE: Ages 60 to 80 RISK FACTORS: Hypertension, atherosclerosis, and family history of aortic aneurysms. Others include inflammatory diseases that cause a vasculitis, disorders of collagen (Marfan syndrome, EhlersDanlos syndrome), bicuspid aortic valve, aortic coarctation, Turner’s syndrome, crack cocaine, and trauma.

CLASSIFICATION Based on the fact that the majority of aortic dissections originate in the ascending or descending aorta, three major classifications (Fig. 1-1): • DeBakey type I ascending and descending aorta, II ascending aorta, III descending aorta • Stanford type A ascending aorta (proximal), type B descending aorta (distal)

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Sudden onset of very severe chest pain, at its peak at onset • Little radiation to neck, shoulder, or arm • Sharp, tearing or ripping pain • Ascending aortic dissection with anterior chest pain • Descending aortic dissection with back pain • Syncope, abdominal pain, CHF, malperfusion may occur • Most with severe hypertension, 25% with hypotension (SBP 100), which can indicate bleeding, cardiac tamponade, or severe aortic regurgitation • Pulse and blood pressure differentials common (38%) caused by partial compression of subclavian arteries • Cardiac and neurological systems are most commonly involved organ systems • Aortic regurgitation in 18% to 50% of cases of proximal dissection • Myocardial ischemia caused by coronary artery compression • Cerebral ischemia/stroke in 5% to 10% of patients • Rare cause of pericarditis

ETIOLOGY • Unknown, risk factors known. Chronic HTN affects arterial wall composition • Medial degeneration of aorta appears to be the culprit • Aortic dissection reflects systemic illness of vasculature

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Known as the great imitator: PE, ACS, AS, pericarditis, cholecystitis • Acute MI needs to be ruled out • Aortic insufficiency • Nondissecting aortic aneurysm

LABORATORY TESTS ECG: Helpful to rule out MI, generally nonspecific findings. Serum biochemical marker: Smooth muscle myosin heavy chain high first 6 hr after onset.

IMAGING STUDIES • Chest x-ray may show widened mediastinum (62%) nonspecific, and displacement of aortic intimal calcium. • Transesophageal echocardiography, sensitivity 97% to 100%, can detect aortic insufficiency and pericardial effusion; study of choice in unstable patients, but operator dependent. • MRI, sensitivity 90% to 100%, gold standard, but length of test and difficult access not suitable for stable intubated patients. Gives best information for surgeons. • CT, sensitivity 83% to 100%, involves IV contrast. • Aortography rarely done now. • Transthoracic echocardiography, poor sensitivity.

• Endovascular stent placement is a new treatment, especially for older highrisk surgical patients.

CHRONIC Rx Chronic aortic dissection (2 wks) followed with aggressive BP control

DISPOSITION • Natural history of untreated aortic dissection is 85% mortality within 2 wk. • Proximal aortic dissection is a surgical emergency. Time is critical; mortality is 1% to 3% per hr. • Patients who are postsurgical repair or have chronic aneurysm should be followed with imaging at 1, 3, 6, 9, and 12 months. • Overall, in-hospital mortality is 30% in patients with proximal dissections and 10% in patients with distal dissections.

REFERRAL For ICU management and surgery

SUGGESTED READINGS Hagan PG et al: The international registry of acute aortic dissection: new insights into an old disease, JAMA 283:897, 2000. Khan IA et al: Clinical, diagnostic, and management perspectives of aortic dissection, Chest 122(1):311, 2002. Moore AG et al: Choice of CT, TEE, MRI and aortography in acute AD: IRAD, Am J Cardiology 89:1235, 2002. Nienaber CA: Aortic dissection: new frontiers in diagnosis and management, Part I and II, Circulation, 108(6):772, 2003. AUTHOR: LYNN BOWLBY, M.D.

TREATMENT ACUTE GENERAL Rx • Admit to ICU for hemodynamic monitoring. • Propanolol 1 mg every 3-5 minutes or metoprolol 5 mg IV every 5 minutes, followed by nitroprusside 0.3-10 mg/ kg/min, with target SBP 100-120. • Decrease contractility and BP with IV -blocker, -blocker is cornerstone of treatment. • IV labetalol can be used instead, 20 mg IV, then 40-80 mg every 10 min. • IV calcium channel blockers or ACE inhibitors may be used. • Proximal dissections require emergent surgery (Type I, II, + A) to prevent rupture or pericardial effusion. • Distal dissections are treated only medically unless distal organ involvement or impending rupture occurs (Type III + B).

Type A or Proximal

Type B or Distal

FIGURE 1-1 Classification systems for aortic dissection. (From Isselbacher EM, Eagle KA, DeSanctis RW: Disease of the aorta. In Braunwald E [ed]: Heart disease: a textbook of cardiovascular medicine, ed 5, Philadelphia, 1997, WB Saunders.)

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Aortic Regurgitation BASIC INFORMATION Aortic regurgitation is retrograde blood flow into the left ventricle from the aorta secondary to incompetent aortic valve. Aortic insufficiency AI AR ICD-9CM CODES 424.1 Aortic valve disorders

EPIDEMIOLOGY & DEMOGRAPHICS • The most common cause of isolated severe aortic regurgitation is aortic root dilation. • Infectious endocarditis is the most frequent cause of acute aortic regurgitation.

PHYSICAL FINDINGS & CLINICAL PRESENTATION The clinical presentation varies depending on whether aortic insufficiency is acute or chronic. Chronic aortic insufficiency is well tolerated (except when secondary to infective endocarditis), and the patients remain asymptomatic for years. Common manifestations after significant deterioration of left ventricular function are dyspnea on exertion, syncope, chest pain, and CHF. Acute aortic insufficiency manifests primarily with hypotension caused by a sudden fall in cardiac output. A rapid rise in left ventricular diastolic pressure results in a further decrease in coronary blood flow. Physical findings in chronic aortic insufficiency include the following: • Widened pulse pressure (markedly increased systolic blood pressure, decreased diastolic blood pressure) is present. • Bounding pulses, head “bobbing” with each systole (de Musset’s sign) are present; “water hammer” or collapsing pulse (Corrigan’s pulse) can be palpated at the wrist or on the femoral arteries (“pistol shot” femorals) and is caused by rapid rise and sudden collapse of the arterial pressure during late systole; capillary pulsations (Quincke’s pulse) may occur at the base of the nail beds. • A to-and-fro “double Duroziez” murmur may be heard over femoral arteries with slight compression. • Popliteal systolic pressure is increased over brachial systolic pressure 40 mm Hg (Hill’s sign). • Cardiac auscultation reveals: 1. Displacement of cardiac impulse downward and to the patient’s left 2. S3 heard over the apex

ETIOLOGY • • • • • • • • • • •

Infective endocarditis Rheumatic fibrosis Trauma with valvular rupture Congenital bicuspid aortic valve Myxomatous degeneration Syphilitic aortitis Rheumatic spondylitis SLE Aortic dissection Fenfluramine, dexfenfluramine Takayasu’s arteritis, granulomatous arteritis

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Patent ductus arteriosus, pulmonary regurgitation, and other valvular abnormalities • The differential diagnosis of cardiac murmurs is described in Section II

WORKUP • Echocardiogram, chest x-ray, ECG, and cardiac catheterization (selected patients) • Medical history and physical examination focused on the following clinical manifestations: 1. Dyspnea on exertion 2. Syncope 3. Chest pain 4. CHF

IMAGING STUDIES • Chest x-ray 1. Left ventricular hypertrophy (chronic aortic regurgitation) 2. Aortic dilation 3. Normal cardiac silhouette with pulmonary edema: possible in patients with acute aortic regurgitation • ECG: left ventricular hypertrophy • Echocardiography: coarse diastolic fluttering of the anterior mitral leaflet; LVH in patients with chronic aortic regurgitation • Cardiac catheterization: assesses degree of left ventricular dysfunction, confirms the presence of a wide pulse pressure, assesses surgical risk, and determines if there is coexistent coronary artery disease

NONPHARMACOLOGIC THERAPY • Avoidance of competitive sports and strenuous activity • Salt restriction

ACUTE GENERAL Rx MEDICAL: • Digitalis, diuretics, ACE inhibitors, and sodium restriction for CHF; nitroprusside in patients with acute aortic regurgitation • Long-term vasodilator therapy with ACE inhibitors or nifedipine for reducing or delaying the need for aortic valve replacement in asymptomatic patients with severe aortic regurgitation and normal left ventricular function • Bacterial endocarditis prophylaxis for surgical and dental procedures SURGICAL: Reserved for: • Symptomatic patients with chronic aortic regurgitation despite optimal medical therapy • Patients with acute aortic regurgitation (i.e., infective endocarditis) producing left ventricular failure • Evidence of systolic failure: 1. Echocardiographic fractional shortening 25% 2. Echocardiographic and diastolic dimension 55 mm 3. Angiographic ejection fraction 50% or end-systolic volume index (ESVI) 60 ml/m2 • Evidence of diastolic failure: 1. Pulmonary pressure 45 mm Hg systolic 2. Left ventricular end-diastolic pressure (LVEDP) 15 mm Hg at catheterization 3. Pulmonary hypertension detected on examination • In general, the “55 rule” has been used to determine the timing of surgery: surgery should be performed before EF 55% or end-systolic dimension 55 mm.

REFERRAL Surgical referral (see “Acute General Rx” for indications)

PEARLS & CONSIDERATIONS COMMENTS The operative mortality rate for aortic regurgitation is 3% to 5%. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

SYNONYMS

TREATMENT Section I

DEFINITION

3. Decrescendo, blowing diastolic murmur heard along left sternal border 4. Low-pitched apical diastolic rumble (Austin-Flint murmur) caused by contrast of the aortic regurgitant jet with the left ventricular wall 5. Early systolic apical ejection murmur In patients with acute aortic insufficiency both the wide pulse pressure and the large stroke volume are absent. A short blowing diastolic murmur may be the only finding on physical examination.

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Aortic Stenosis BASIC INFORMATION DEFINITION Aortic stenosis is obstruction to systolic left ventricular outflow across the aortic valve. Symptoms appear when the valve orifice decreases to 1 cm2 (normal orifice is 3 cm2). The stenosis is considered severe when the orifice is 0.5 cm2/m2 or the pressure gradient is 50 mm Hg or higher.

SYNONYMS Aortic valvular stenosis AS ICD-9CM CODES 424.1 Aortic valvular stenosis

EPIDEMIOLOGY & DEMOGRAPHICS • Aortic stenosis is the most common valve lesion in adults in Western countries. • Calcific stenosis (most common cause in patients 60 yr old) occurs in 75% of patients.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Rough, loud systolic diamond-shaped murmur, best heard at base of heart and transmitted into neck vessels; often associated with a thrill or ejection click; may also be heard well at the apex • Absence or diminished intensity of sound of aortic valve closure (in severe aortic stenosis) • Late, slow-rising carotid upstroke with decreased amplitude • Strong apical pulse • Narrowing of pulse pressure in later stages of aortic stenosis • Some patients with aortic stenosis experience bleeding into their GI tract or skin. This is caused by an acquired defect in von Willebrand factor. Aortic valve replacement restores normal hemostasis.

ETIOLOGY • Rheumatic inflammation of aortic valve • Progressive stenosis of congenital bicuspid valve (found in 1%-2% of population) • Idiopathic calcification of the aortic valve • Congenital (major cause of aortic stenosis in patients 30 yr)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Hypertrophic cardiomyopathy • Mitral regurgitation • Ventricular septal defect

• Aortic sclerosis. Aortic stenosis is distinguished from aortic sclerosis by the degree of valve impairment. In aortic sclerosis, the valve leaflets are abnormally thickened but obstruction to outflow is minimal.

WORKUP • Echocardiography • Chest x-ray examination, ECG • Cardiac catheterization in selected patients (see “Imaging Studies”) • Medical history focusing on symptoms and potential complications: 1. Angina 2. Syncope (particularly with exertion) 3. CHF 4. GI bleeding: in patients with associated hemorrhagic telangiectasia (AVM)

IMAGING STUDIES • Chest x-ray examination 1. Poststenotic dilation of the ascending aorta 2. Calcification of aortic cusps 3. Pulmonary congestion (in advanced stages of aortic stenosis) • ECG: 1. Left ventricular hypertrophy (found in 80% of patients) 2. ST-T wave changes 3. Atrial fibrillation: frequent • Doppler echocardiography: thickening of the left ventricular wall; if the patient has valvular calcifications, multiple echoes may be seen from within the aortic root and there is poor separation of the aortic cusps during systole. Gradient across the valve can be estimated but is less precise than with cardiac catheterization. • Cardiac catheterization: indicated in symptomatic patients; it confirms the diagnosis and estimates the severity of the disease by measuring the gradient across the valve, allowing calculation of the valve area. It also detects coexisting coronary artery stenosis that may need bypass at the same time as aortic valve replacement.

TREATMENT NONPHARMACOLOGIC THERAPY • Strenuous activity should be avoided. • Sodium restriction if CHF is present.

GENERAL Rx MEDICAL: • Diuretics and sodium restriction are needed if CHF is present; digoxin is used only to control rate of atrial fibrillation. • ACE inhibitors are relatively contraindicated.

• Calcium channel blocker verapamil may be useful only to control rate of atrial fibrillation. • Antibiotic prophylaxis is necessary for surgical and dental procedures. SURGICAL: • Valve replacement is the treatment of choice in symptomatic patients because the 5-yr mortality rate after onset of symptoms is extremely high, even with optimal medical therapy; valve replacement is indicated if cardiac catheterization establishes a pressure gradient 50 mm Hg and valve area 1 cm2. • Balloon aortic valvotomy for adult acquired aortic stenosis is useful only for palliation.

DISPOSITION • The 5-yr survival rate in adults is 40%. • The average duration of symptoms before death is as follows: angina, 60 mo; syncope, 36 mo; CHF, 24 mo. • About 75% of patients with symptomatic aortic stenosis will be dead 3 yr after onset of symptoms unless the aortic valve is replaced.

REFERRAL • Surgical referral for valve replacement in symptomatic patients. However, the presence of moderate or severe valvular calcification, together with a rapid increase in aortic-jet velocity, identifies patients with a very poor prognosis who should be considered for early valve replacement rather than have surgery delayed until symptoms develop. • Surgical mortality rate for valve replacement is 3% to 5%; however, it varies with patient’s age (8% in patients 75 yr old). • Balloon valvuloplasty is useful in poor surgical candidates who do not have calcified valve apparatus; it can be done as an intermediate procedure to stabilize high-risk patients before surgery. • When performed in adults who have calcified valves, balloon valvuloplasty is useful only for short-term reduction in severity of aortic stenosis when surgery is contraindicated, because restenosis occurs rapidly.

SUGGESTED READINGS Alpert JS: Aortic stenosis, a new face for an old disease, Arch Intern Med 163:1769, 2003. Carabello BA: Aortic stenosis, N Engl J Med 346:677, 2002. Vincentelli A et al: Acquired von Willebrand syndrome in aortic stenosis, N Engl J Med 349:343, 2003. AUTHOR: FRED F. FERRI, M.D.

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Arthritis, Rheumatoid BASIC INFORMATION

ICD-9CM CODES 714.0 Rheumatoid arthritis

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 5 cases/1000 adults PREDOMINANT SEX: • Female:male ratio of 3:1 • After age 50 yr, sex difference less marked

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Usually gradual onset; common prodromal symptoms of weakness, fatigue, and anorexia • Initial presentation: multiple symmetric joint involvement, most often in the hands and feet, usually MCP, MTP, and PIP joints (Fig. 1-2) • Joint effusions, tenderness, and restricted motion usually present early in the disease • Eventual characteristic deformities: subluxations, dislocations, and joint contractures • Extraarticular findings: 1. Tendon sheaths and bursae frequently affected by chronic inflammation 2. Possible tendon rupture 3. Rheumatoid nodules over bony prominences such as the elbow and shaft of the ulna

ETIOLOGY Unknown. There is increasing evidence that the inflammation and destruction of bone and cartilage that occur in many rheumatic diseases are the result of the activation by some unknown mechanism of proinflammatory cells that infiltrate the synovium. These cells, in turn, release various substances, such as cytokines and tumor necrosis factor (TNF) alpha, which subsequently cause the pathologic changes typical of this group of diseases. Many of the newer therapeutic agents are directed at the suppression of these final mediators of inflammation.

• Increase in rheumatoid factor in 80% of cases (rheumatoid factor also present in the normal population) • Possible mild anemia • Usually, elevated acute phase reactants (ESR, C-reactive protein) • Possible mild leukocytosis • Usually, turbid joint fluid, which forms a poor mucin clot; elevated cell count, with an increase in polymorphonuclear leukocytes

IMAGING STUDIES Plain radiography • Usually reveals soft-tissue swelling and osteoporosis early (Fig. 1-3) • Eventually, joint space narrowing, erosion, and deformity visible as a result of continued inflammation and cartilage destruction

DIAGNOSIS

TREATMENT

DIFFERENTIAL DIAGNOSIS

NONPHARMACOLOGIC THERAPY

• SLE • Seronegative spondyloarthropathies • Polymyalgia rheumatica • Acute rheumatic fever • Scleroderma According to the American College of Rheumatology, RA exists when four of seven criteria are present, with criteria 1 to 4 being present for at least 6 wk. 1. Morning stiffness over 1 hr 2. Arthritis in three or more joints with swelling 3. Arthritis of hand joints with swelling 4. Symmetric arthritis 5. Rheumatoid nodules 6. Roentgenographic changes typical of RA 7. Positive serum rheumatoid factor

Proper management requires close cooperation among primary physician, therapist, rheumatologist, and orthopedist. • Patient education is important. • Rest with proper exercise and splinting can prevent or correct joint deformities. • Maintain proper diet and control obesity.

CHRONIC Rx • NSAIDs: commonly used as the initial treatment to relieve inflammation (drug of choice for most patients: aspirin, but other NSAIDs also effective) • Disease-modifying drugs (DMARDs): are traditionally begun when NSAIDs

FIGURE 1-2 Rheumatoid arthritis. Hand of a 60-year-old man with seropositive rheumatoid arthritis. There are fixed deformities and gross rheumatoid nodules. (From Canoso JJ: Rheumatology in primary care, Philadelphia, 1997, WB Saunders.)

DISEASES AND DISORDERS

Rheumatoid arthritis (RA) is a systemic disorder characterized by chronic joint inflammation that most commonly affects peripheral joints. This process results in the development of pannus, a destructive tissue that damages cartilage.

LABORATORY TESTS Section I

DEFINITION

4. Splenomegaly, pericarditis, and vasculitis 5. Findings of carpal tunnel syndrome resulting from flexor tenosynovitis

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Arthritis, Rheumatoid are not effective; current recommendations favor early aggressive treatment with DMARDs, seeking to minimize long-term joint damage. Commonly used agents are methotrexate, cyclosporine, hydroxychloroquine, sulfasalazine, leflunomide, adalimumab and infliximab. Most of these are associated with potential toxicity and require close monitoring. They are also usually slow-acting drugs that require more than 8 wk to become effective. • Oral prednisone • Intrasynovial steroid injections • Etanercept (Enbrel), a tumor necrosis factor -blocker, is indicated in moderately to severely active RA in patients who respond inadequately to DMARDs. The combination of etanercept and methotrexate has been reported to be effective and promising in the treatment of RA.

DISPOSITION • Remissions and exacerbations are common, but condition is chronically progressive in the majority of cases.

• Joint degeneration and deformity often lead to disability. • Early diagnosis and treatment are important and can improve quality of life.

REFERRAL Early referral to rheumatologist Orthopedic consultation for corrective surgery

SUGGESTED READINGS Chen AL, Joseph TN, Zuckerman JD: Rheumatoid arthritis of the shoulder, J Am Acad Orthop Surg 11:12, 2003. Cohen S et al: Treatment of rheumatoid arthritis with anakinra, a recombinant human interleukin-1 receptor antagonist, in combination with methotrexate: results of a twenty-four-week, multicenter, randomized, double-blind, placebo controlled trial, Arthritis Rheum 46:614, 2002. Dayer J-M, Bresnihan B: Targeting interleukin1 in the treatment of rheumatoid arthritis, Arthritis Rheum 46:574, 2002. Edwards JC, Szczepanski L et al: Efficacy of cell-targeted therapy with ritaximab in patients with rheumatoid arthritis, N Engl J Med 350:2572, 2004.

Gardner GC, Kadel MJ: Ordering and interpreting rheumatologic laboratory tests, J Am Acad Orthop Surg 11:60, 2003. Genovese MC et al: Etanercept versus methotrexate in patients with early rheumatoid arthritis: two-year radiographic and clinical outcomes, Arthritis Rheum 46:1443, 2002. Kremer JM: Rational use of new and existing disease-modifying agents in rheumatoid arthritis, Ann Intern Med 134:695, 2001. Olsen NJ, Stein CM: New drugs for rheumatoid arthritis, N Engl J Med 350:2167, 2004. Maini SR: Infliximab treatment of rheumatoid arthritis, Rheum Dis Clin North Am 30:329, 2004. Smith JB, Haynes MK: Rheumatoid arthritis: a molecular understanding, Ann Intern Med 136:908, 2002. Van Everdingen AA et al: Low dose prednisone therapy for patients with early active rheumatoid arthritis: clinical efficacy, disease-modifying properties, and side effects, Ann Intern Med 136:1, 2002. AUTHOR: LONNIE R. MERCIER, M.D.

B

A

FIGURE 1-3 Rheumatoid arthritis. A, Periarticular osteopenia and marginal erosions in MCPs and a PIP (arrows). B, In the same patient, marginal erosions at metatarsal heads. (From Canoso JJ [ed]: Rheumatology in primary care, Philadelphia, 1997, WB Saunders.)

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Aseptic Necrosis BASIC INFORMATION Cell death in components of bone: hematopoietic fat marrow and mineralized tissue. Osteonecrosis, avascular necrosis ICD-9CM CODES 733.40 Aseptic necrosis 733.43 Aseptic necrosis condyle 733.42 Aseptic necrosis head 733.41 Aseptic necrosis head 733.44 Aseptic necrosis

of femoral of femoral of humeral of talus

EPIDEMIOLOGY & DEMOGRAPHICS • 15,000 new cases per year in the U.S. • Associated conditions: 1. Corticosteroid treatment: 35% 2. Alcohol abuse: 22% 3. Idiopathic and other: 43% • Common sites involved 1. Femoral head 2. Femoral condyle 3. Humeral head 4. Navicular and lunate wrist bones 5. Talus

PHYSICAL FINDINGS & CLINICAL PRESENTATION • May be asymptomatic • Pain in the involved area exacerbated by movement or weight bearing • Decreased range of motion as the disease progresses • Functional limitation

A

B

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • None in late stages • Early: any condition causing focal musculoskeletal pain including arthritis, bursitis, tendinitis, myopathy, neoplastic bone and joint diseases, traumatic injuries, pathologic fractures

IMAGING STUDIES See Fig. 1-4. 1. X-ray: insensitive early in the course. The earliest changes include diffuse osteopenia, areas of radiolucency with sclerotic border, and linear sclerosis. Later a subchondral lucency (crescent sign) indicates subchondral fracture. More advanced cases reveal flattening, collapsed bone and abnormal bone contour. In late disease, osteoarthritic changes are seen.

2. Bone scan: • Early: “cold” area • Later: increased radionuclide uptake as a result of remodeling • Sensitivity in early disease is only 70% and specificity is poor 3. CT scan: may reveal central necrosis and area of collapse before those are visible in x-ray. 4. MRI: the most sensitive technology to diagnose early aseptic necrosis. The first sign is a margin of low signal. An inner border of high signal associated with a low-signal line is specific of aseptic necrosis (“double line sign”). Sensitivity is 75%-100%.

TREATMENT PREVENTION • Management of etiologic conditions • Minimize corticosteroid use

MEDICAL TREATMENT • Decrease weight bearing of affected area • Pulsing electromagnetic fields applied externally (still experimental) • Peripheral vasodilators (e.g., dihydroergotamine) (unproven)

SURGICAL TREATMENT • Core decompression: effectiveness 35%-95% in early phases • Bone grafting • Osteotomies • Joint replacement

PROGNOSIS • When diagnosed at an early stage treatment is appropriate in all cases because 85%-90% can be expected to progress to a more advanced stage • Contralateral joint involvement is common (30%-70%)

SUGGESTED READING Mazieres R: Osteonecrosis: In Klippel JH, Dieppe PA (eds): Rhematology, ed 2, St. Louis, 1998, Mosby. AUTHOR: TOM J. WACHTEL, M.D.

FIGURE 1-4 Aseptic necrosis of the hips. A, Aseptic necrosis can occur from a number of causes, including trauma and steroid use. In this patient, an anteroposterior view of the pelvis shows a transplanted kidney (K) in the right iliac fossa. Use of steroids has caused this patient to have bilateral aseptic necrosis. The femoral heads are somewhat flattened, irregular, and increased in density. B, Aseptic necrosis in a different patient is demonstrated on an MRI scan as an area of decreased signal (arrows) in the left femoral head. This is the most sensitive method for detection of early aseptic necrosis. (From Mettler FA [ed]: Primary care radiology, Philadelphia, 2000, WB Saunders).

DISEASES AND DISORDERS

SYNONYMS

Final common pathway of conditions that lead to impairment of the blood supply to the involved bone. Stages: Stage 0 • Asymptomatic • Normal imaging • Histologic findings only (i.e., silent osteonecrosis) Stage 1 • Asymptomatic or symptomatic • Normal x-ray and CT scan • Abnormal bone scan and/or MRI Stage 2 • Abnormal x-rays and/or CT scan including linear sclerosis, focal bead mineralization, cysts; however, the overall architecture of the involved bone is normal Stage 3 • Early evidence of mechanical bone failure (subchondral fracture), but the overall shape of the bone is still intact Stage 4 • Flattening or collapse of the bone Stage 5 • Joint space narrowing Stage 6 • Extensive joint destruction

Section I

DEFINITION

ETIOLOGY

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Asthma BASIC INFORMATION DEFINITION The American Thoracic Society defines asthma as a “disease characterized by an increased responsiveness of the trachea and bronchi to various stimuli and manifested by a widespread narrowing of the airways that changes in severity either spontaneously or as a result of treatment.” Status asthmaticus can be defined as a severe continuous bronchospasm.

SYNONYMS Bronchospasm Reactive airways disease Bronchial asthma ICD-9CM CODES 493.9 Asthma, unspecified 493.1 Intrinsic asthma 493.0 Extrinsic asthma

EPIDEMIOLOGY & DEMOGRAPHICS • Asthma affects 5% to 12% of the population and accounts for over 450,000 hospitalizations and nearly 2 million emergency department visits yearly in the U.S. • Overall asthma mortality in the U.S. is 20 per 1 million persons per year. • In older individuals, asthma and COPD may overlap.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Physical examination varies with the stage and severity of asthma and may reveal only an increased expiratory phase of respiration. The triad of typical asthma symptoms includes shortness of breath, wheezing, and cough. The hallmark physical finding in any patient with active asthma is wheezing (expiratory  inspiratory) and a prolonged expiratory phase during lung auscultation. Physical examination during status asthmaticus may reveal: • Tachycardia and tachypnea • Use of accessory respiratory muscles • Pulsus paradoxus (inspiratory decline in systolic blood pressure 10 mm Hg) • Wheezing: absence of wheezing (silent chest) or decreased wheezing can indicate worsening obstruction • Mental status changes: generally secondary to hypoxia and hypercapnia and constitute an indication for urgent intubation • Paradoxic abdominal and diaphragmatic movement on inspiration (detected by palpation over the upper part of the abdomen in a semirecumbent position): important sign of impending respiratory crisis; indicates diaphragmatic fatigue

• The following abnormalities in vital signs are indicative of severe asthma: 1. Pulsus paradoxus 18 mm Hg 2. Respiratory rate 30 breaths/min 3. Tachycardia with heart rate 120 beats/min

ETIOLOGY • Intrinsic asthma: occurs in patients who have no history of allergies; may be triggered by upper respiratory infections or psychologic stress • Extrinsic asthma (allergic asthma): brought on by exposure to allergens (e.g., dust mites, cat allergen, industrial chemicals) • Exercise-induced asthma: manifests with bronchospasm following initiation of exercise and improves with discontinuation of exercise • Drug-induced asthma: often associated with use of NSAIDs, beta blockers, sulfites, certain foods and beverages

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • • • • •

CHF COPD Pulmonary embolism Foreign body aspiration Pneumonia and other upper respiratory infections Rhinitis with postnasal drip TB Hypersensitivity pneumonitis Anxiety disorder Wegener’s granulomatosis Diffuse interstitial lung disease GERD

WORKUP Medical history, physical examination, pulmonary function studies and peak flow meter determination, blood gas analysis and oximetry (during acute bronchospasm), chest radiography if infection is suspected

LABORATORY TESTS Laboratory tests can be normal if obtained during a stable period. The following laboratory abnormalities may be present during an acute bronchospasm: • ABGs can be used in staging the severity of an asthmatic attack: 1. Mild: decreased Pao2 and Paco2, increased pH 2. Moderate: decreased Pao2, normal Paco2, normal pH 3. Severe: marked decreased Pao2, increased Paco2, and decreased pH • CBC, leukocytosis with “left shift” may indicate the existence of bacterial infection.

• Sputum: eosinophils, Charcot-Leyden crystals, PMNs, and bacteria may be found on Gram stain in patients with pneumonia. • Useful diagnostic tests for asthma: 1. Pulmonary function studies: during acute severe bronchospasm, FEV1 is 1 L and peak expiratory flow rate (PEFR) is 80 L/min 2. Methacholine challenge test 3. Skin test: to assess the role of atopy (when suspected)

IMAGING STUDIES • Chest x-ray: usually normal, may show evidence of thoracic hyperinflation (e.g., flattening of the diaphragm, increased volume over the retrosternal air space) • ECG: tachycardia, nonspecific ST-T wave changes; may also show cor pulmonale, right bundle-branch block, right axial deviation, counter-clockwise rotation

TREATMENT NONPHARMACOLOGIC THERAPY • Avoidance of triggering factors (e.g., salicylates, sulfites). • Encouragement of regular exercise (e.g., swimming). • Patient education regarding warning signs of an attack and proper use of medications (e.g., correct use of inhalers). Elderly asthmatics must be observed using the handheld inhalers. A spacer may be helpful and should be ordered routinely. If they cannot be taught to use metered-dose inhalers, they should be prescribed nebulizers for routine use at home. Albuterol, ipratropium, and fluticasone are available for nebulizer use.

GENERAL Rx The Expert Panel of the National Asthma Education and Prevention Program (NAEPP) based on the classification of asthma severity recommends the following stepwise approach in the pharmacologic management of asthma in adults: STEP 1 (MILD INTERMITTENT ASTHMA): NO DAILY MEDICATIONS ARE NEEDED. • Short-acting inhaled 2-agonists as needed (e.g., albuterol). STEP 2 (MILD PERSISTENT ASTHMA): DAILY TREATMENT MAY BE NEEDED. • Low-dose inhaled corticosteroid (e.g., beclomethasone [Beclovent, Vanceril], flunisolide [AeroBid], triamcinolone [Azmacort]) or fluticasone (Flovent) can be used. • Cromolyn (Intal) or nedocromil (Tilade) can also be used.

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Asthma COMMENTS • Inhaled low-dose corticosteroids are the single most effective therapy for adult patients with asthma who require more than an occasional use of short-acting 2-agonists to control their asthma. • Leukotriene modifiers/receptor agonists represent a reasonable alternative in adults unable or unwilling to use corticosteroids; however, these agents are less effective than monotherapy with inhaled corticosteroids. • Patients who remain symptomatic despite inhaled corticosteroids benefit from the addition of long-acting 2-agonists. • In patients with allergies and elevated serum IgE levels, use of anti-IgE therapy is beneficial.

SUGGESTED READINGS

REFERRAL

Diette GB et al: Asthma in older patients: factors associated with hospitalization, Arch Intern Med 162:1123, 2002. Holgate ST: Therapeutic options for persistent asthma, JAMA 285:2637, 2001. Mintz M: Asthma update: part I. Diagnosis, monitoring, and prevention of disease progression, Am Fam Physician 70:893-898, 2004. National Asthma Education and Prevention Program: Expert panel report 2: guidelines for diagnosis and management of asthma, Bethesda, Md, 1997, National Institutes of Health. Naureckas ET, Solway J: Mild asthma, N Engl J Med 345:1257, 2001. Sin DD et al: Pharmacological management to reduce exacerbations in adults with asthma, JAMA 292:367-376, 2004.

Box 1-1 describes indications for referral to an asthma specialist.

AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

• •

•

•

BOX 1-1

Possible Indications for Referral to an Asthma Specialist

Severe, acute asthma that has caused loss of consciousness, hypoxia, respiratory failure, convulsions, or near death Poorly controlled asthma as indicated by admission to a hospital, frequent need for emergency care, need for oral corticosteroids, absence from work, disruption of sleep, interference with quality of life Severe, persistent asthma requiring step 4 care (consider for patients who require step 3 care) Requirement for continuous oral corticosteroids or high-dose inhaled corticosteroids or more than two short courses of oral corticosteroids within 1 year Need for additional diagnostic testing such as allergy skin testing, rhinoscopy, provocative challenge, complete pulmonary function testing, bronchoscopy Consideration for immunotherapy Need for additional education regarding asthma, complications of asthma and treatment of asthma, problems with adherence to management recommendations, or allergen avoidance Uncertainty of diagnosis Complications of asthma, including sinusitis, nasal polyposis, aspergillosis, severe rhinitis, vocal cord dysfunction, gastroesophageal reflux Modified from National Asthma Education and Prevention Program, National Heart, Lung, and Blood Institute, Expert Panel Report 2: Guidelines for the diagnosis and management of asthma. Washington, DC, NIH Pub No 97-4051, July 1997.

DISEASES AND DISORDERS

(Solu-Medrol) 0.5 to 1 mg/kg IV loading dose, then q6h prn; higher doses may be necessary in selected patients (particularly those receiving steroids at home); steroids given by inhalation should be (re)initiated after the acute phase, are useful for controlling bronchospasm and tapering oral steroids, and should be used in all patients with severe asthma. 3. Rapid but judicious tapering of corticosteroids will eliminate serious steroid toxicity; long-term low-dose methotrexate may be an effective means of reducing the systemic corticosteroid requirement in some patients with severe refractory asthma. 4. The most common errors regarding steroid therapy in acute bronchospasms are the use of “too little, too late” and too rapid tapering with return of bronchospasm. IV hydration: judicious use is necessary to avoid CHF in elderly patients. IV antibiotics are indicated when there is suspicion of bacterial infection (e.g., infiltrate on chest x-ray, fever, or leukocytosis). Intubation and mechanical ventilation are indicated when previous measures fail to produce significant improvement. General anesthesia: halothane may reverse bronchospasm in a severe asthmatic who cannot be ventilated adequately by mechanical means.

Section I

• Additional considerations for longterm control are the use of the leukotriene receptor antagonist montelukast (Singulair). • Quick relief of asthma can be achieved with short-acting inhaled 2-agonists (see Step 1) (often described as “rescue treatment”). STEP 3 (MODERATE PERSISTENT ASTHMA): DAILY MEDICATION IS RECOMMENDED. • Low-dose or medium-dose inhaled corticosteroids (see Step 2) plus longacting inhaled 2-agonist (salmeterol [Serevent]), or long-acting oral 2-agonists (e.g., albuterol, sustained-release tablets). Salmeterol is also available as a dry powder inhaler (Discus) that does not require a spacer device; the dosage is one puff bid. A salmeterolfluticasone combination for the Discus inhaler (Advair) is available and simplifies therapy for patients with asthma. It generally should be reserved for patients with at least moderately severe asthma not controlled by an inhaled corticosteroid alone. • Use short-acting inhaled 2-agonists on a prn basis for quick relief. STEP 4 (SEVERE PERSISTENT ASTHMA): • Daily treatment with high-dose inhaled corticosteroids plus long-acting inhaled -agonists plus long-acting oral 2-agonist plus long-term systemic corticosteroids (e.g., methylprednisolone, prednisolone, prednisone) can be used. • Short-acting 2-agonists can be used on a prn basis for quick relief. Treatment of status asthmaticus is as follows: • Oxygen generally started at 2 to 4 L/min via nasal cannula or Venti-Mask at 40% Fio2; further adjustments are made according to the ABGs. • Bronchodilators: various agents and modalities are available. Inhaled bronchodilators are preferred when they can be administered quickly. Albuterol (Proventil, Ventolin): 0.5 to 1 ml (2.5 to 5 mg) in 3 ml of saline solution tid or qid via nebulizer is effective. Parenteral administration of sympathomimetics (e.g., SC epinephrine) is not recommended routinely and should always be accompanied by electrocardiographic monitoring. • Corticosteroids 1. Early administration is advised, particularly in patients using steroids at home. 2. Patients may be started on hydrocortisone (Solu-Cortef) 2.5 to 4 mg/kg or methylprednisolone

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Atrial Fibrillation

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BASIC INFORMATION DEFINITION Atrial fibrillation is totally chaotic atrial activity caused by simultaneous discharge of multiple atrial foci.

SYNONYMS AF A-fib ICD-9CM CODES 427.31 Atrial fibrillation

EPIDEMIOLOGY & DEMOGRAPHICS The prevalence of atrial fibrillation increases with age, from 2% in the general population to 5% in patients older than 60 yr, and 9% of those aged 80 years or older.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Clinical presentation is variable: • Asymptomatic patients • Most common complaint: palpitations • Fatigue, dizziness, light-headedness in some patients • CHF • Cardiac auscultation revealing irregularly irregular rhythm

ETIOLOGY • • • • • • • • • • •

Coronary artery disease MS, MR, AS, AR Thyrotoxicosis Pulmonary embolism, COPD Pericarditis Myocarditis, cardiomyopathy Tachycardia-bradycardia syndrome Alcohol abuse MI WPW syndrome Other causes: left atrial myxoma, atrial septal defect, carbon monoxide poisoning, pheochromocytoma, idiopathic hypoxia, hypokalemia, sepsis, pneumonia

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Multifocal atrial tachycardia • Atrial flutter • Frequent atrial premature beats

WORKUP New-onset atrial fibrillation: ECG, echocardiogram, Holter monitor (selected patients), and laboratory evaluation

LABORATORY TESTS • TSH • CBC, glu, creatinine, sodium, potassium, ALT, alkaline, phosphatase, calcium

IMAGING STUDIES • ECG 1. Irregular, nonperiodic wave forms (best seen in V1) reflecting continuous atrial reentry 2. Absence of P waves 3. Conducted QRS complexes showing no periodicity • Echocardiography to evaluate left atrial size and detect valvular disorders in all patients • Holter monitor: useful only in selected patients to evaluate paroxysmal atrial fibrillation

TREATMENT NONPHARMACOLOGIC THERAPY • Avoidance of alcohol in patients with suspected excessive alcohol use • Avoidance of caffeine and nicotine

ACUTE GENERAL Rx • New-onset atrial fibrillation: 1. If the patient is hemodynamically unstable, emergency synchronized cardioversion may be required following immediate conscious sedation with a rapid short-acting sedative (e.g., midazolam). • Cardioversion is indicated if the ventricular rate is 140 bpm and the patient is symptomatic (particularly in acute MI, chest pain, dyspnea, CHF) or when there is no conversion to normal sinus rhythm after 3 days of pharmacologic therapy. The likelihood of cardioversion-related clinical thromboembolism is low in patients with atrial fibrillation lasting 48 hr. Patients with atrial fibrillation lasting 2 days have a 5% to 7% risk of clinical thromboembolism if cardioversion is not preceded by several weeks of warfarin therapy. However, if transesophageal echocardiography reveals no atrial thrombus, cardioversion may be performed safely after only a short period of anticoagulant therapy. Anticoagulant therapy should be continued for at least 1 mo after cardioversion to minimize the incidence of adverse thromboembolic events following conversion from atrial fibrillation to sinus rhythm. 2. If the patient is hemodynamically stable, treatment options include the following in a hospital setting: • Diltiazem 0.25 mg/kg given over 2 min followed by a second dose of 0.35 mg/kg 15 min later if the rate is not slowed. May then fol-

• • •

•

low with intravenous infusion 10 mg/hr (range 5-15 mg/hr). Onset of action following IV administration is usually within 3 min, with peak effect most often occurring within 10 min. After the ventricular rate is slowed, the patient can be changed to oral diltiazem 60 to 90 mg q6h. • Verapamil 2.5 to 5 mg IV initially, then 5 to 10 mg IV 10 min later if the rate is still not slowed. After the ventricular rate is slowed, the patient can be changed to oral verapamil 80 to 120 mg q6-8h. • Esmolol, metoprolol, atenolol are -blockers that are available in IV preparations that can be used in atrial fibrillation. • Other medications useful for converting atrial fibrillation to sinus rhythm are ibutilide, flecainide, propafenone, disopyramide, amiodarone, and quinidine. • Digoxin is not a very potent AV nodal blocking agent and cannot be relied on for acute control of the ventricular response. When used, give 0.5 mg IV loading dose (slow), then 0.25 mg IV 6 hours later. A third dose may be needed after 6 to 8 h; daily dose varies from 0.125 to 0.25 mg (decrease dosage in patients with renal insufficiency and elderly patients). Digoxin should be avoided in WPW patients with atrial fibrillation. Procainamide is the preferred pharmacologic agent in these patients. • Loop diuretics and oxygen are used for CHF. • If the patient is asymptomatic or if the only symptom is palpitation or mild CHF, treatment may be initiated on an outpatient basis with any of the above medications given orally. IV heparin or SC low-molecular-weight heparin for hospitalized patients; sometimes omitted for outpatients. Anticoagulate with warfarin (unless patient has specific contraindications). Long-term anticoagulation with warfarin (adjusted to maintain an INR of 2 to 3) is indicated in all patients with atrial fibrillation; however, the safety of long-term anticoagulation with warfarin in the long-term care setting is not established and should therefore be individualized to the patient and nursing home. Aspirin 325 mg/day may be a suitable alternative to warfarin in patients 70 yr with increased risk of bleeding. The efficacy of clopidogrel (Plavix) is not known.

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Atrial Fibrillation

• Anticoagulation with warfarin (see “Acute General Rx”) • Rate control with digoxin, verapamil, diltiazem, or -blockers

DISPOSITION Factors associated with maintenance of sinus rhythm following cardioversion: • Left atrium diameter 60 mm • Absence of mitral valve disease • Short duration of atrial fibrillation

REFERRAL • Catheter-based radiofrequency ablation procedures designed to eliminate atrial fibrillation represent newer approaches to atrial fibrillation. • Implantable pacemakers and defibrillators that combine pacing and cardioversion therapies to both prevent and treat atrial defibrillation are likely to have an increasing role in the future management of atrial fibrillation.

COMMENTS The American Academy of Family Physicians and the American College of Physicians provide the following recommendations for the management of newly detected atrial fibrillation: 1. Rate control with chronic anticoagulation is the recommended strategy for the majority of patients with atrial fibrillation. Rhythm control has not been shown to be superior to rate control (with chronic anticoagulation) in reducing morbidity and mortality and may be inferior in some patient subgroups to rate control. Rhythm control is appropriate when based on other special considerations, such as patient symptoms, exercise tolerance, and patient preference. 2. Patients with atrial fibrillation should receive chronic anticoagulation with adjusted-dose warfarin, unless they are at low risk of stroke or have a specific contraindication to the use of warfarin (thrombocytopenia, recent trauma or surgery, alcoholism). 3. For patients with atrial fibrillation, the following drugs are recommended for their demonstrated efficacy in rate control during exercise and while at rest: atenolol, metoprolol, diltiazem, and verapamil (drugs listed alphabetically by class). Digoxin is only effective for rate control at rest and therefore should only be used as a second line agent for rate control in atrial fibrillation. 4. For those patients who elect to undergo acute cardioversion to achieve sinus rhythm in atrial fibrillation, both direct-current cardioversion and pharmacologic conversion are appropriate options. 5. Both transesophageal echocardiography with short-term prior anticoagulation followed by early acute cardioversion (in absence of intracardiac thrombus) with postcardioversion anticoagulation and delayed cardioversion with pre- and postanticoagula-

tion are appropriate management strategies for those patients who elect to undergo cardioversion. 6. Most patients converted to sinus rhythm from atrial fibrillation should not be placed on rhythm maintenance therapy because the risks outweigh the benefits. In a selected group of patients whose quality of life is compromised by atrial fibrillation, the recommended pharmacologic agents for rhythm maintenance are amiodarone, disopyramide, propafenone, and sotalol (drugs listed in alphabetical order). The choice of agent depends on specific risk of side effects based on patient characteristics.

SUGGESTED READINGS Cooper JM et al: Implantable devices for the treatment of atrial fibrillation, N Engl J Med 346:2062, 2002. Ezekowitz M, Falk RH: The increasing need for anticoagulation therapy to prevent stroke in patients with atrial fibrillation, Mayo Clin Proc 79(7):904-913, 2004. Hart RG: Atrial fibrillation and stroke prevention, N Engl J Med 349:1015, 2003. Hilek E et al: Effect of intensity of oral anticoagulation on stroke severity and mortality in atrial fibrillation, N Engl J Med 349:1019, 2003. Klein AL et al: Use of transesophageal echocardiography to guide cardioversions in patients with atrial fibrillation, N Engl J Med 344:1411, 2001. Petersen P et al: Ximelagran versus warfarin for stroke prevention in patients with nonvalvular atrial fibrillation. SPORTIF II: A dose-guiding, tolerability, and safety study, J Am Coll Cardiol 41:1445, 2003. Snow V et al: Management of newly detected atrial fibrillation: a clinical practice guideline from the American Academy of Family Physicians and the American College of Physicians, Ann Intern Med 139:1009-1017, 2003. AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

CHRONIC Rx

PEARLS & CONSIDERATIONS

Section I

• Medical cardioversion: 1. Attempts at medical (pharmacologic) intervention should be considered only after proper anticoagulation because cardioversion can lead to systemic emboli. Following successful cardioversion, anticoagulation with warfarin should be continued for 4 wk. 2. Useful agents for medical cardioversion are quinidine, flecainide, propafenone, amiodarone, ibutilide, sotalol, dofetilide, and procainamide. 3. Amiodarone appears to be the most effective agent for converting to sinus rhythm in patients who do not respond to other agents. Amiodarone therapy should be considered for patients with recent atrial fibrillation and structural heart disease, particularly those with left ventricular dysfunction. Amiodarone should also be considered for patients with refractory conditions who do not have heart disease, before therapies with irreversible effects such as AV nodal ablation are attempted.

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Barrett’s Esophagus BASIC INFORMATION DEFINITION Barrett’s esophagus occurs when the squamous lining of the lower esophagus is replaced by metaplastic, intestinalized columnar epithelium. The condition is associated with an increased risk of adenocarcinoma of the esophagus.

SYNONYMS Intestinal metaplasia of the lower esophagus ICD-9CM CODES 530.85 Barrett’s esophagus

EPIDEMIOLOGY & DEMOGRAPHICS • Male predominance with a 4:1 ratio of men to women • Mean age of onset is 40 yr with a mean age of diagnosis of 55 to 60 yr • Occurs more frequently in Caucasians and Hispanics than in African Americans with a ratio of 10-20:1 • Mean prevalence of 5% to 15% in patients undergoing endoscopy for symptoms of GERD

PHYSICAL FINDINGS & CLINICAL PRESENTATION Symptoms: • Typically, chronic (5 yr) heartburn • May be an incidental finding in patients undergoing endoscopy for indications unrelated to GERD • Dysphagia for solid food • Less frequent: chest pain, hematemesis, or melena Physical findings: • Nonspecific • Ranges from epigastric tenderness on palpation to completely normal

ETIOLOGY • Metaplasia is thought to result from reepithelialization of esophageal tissue injured secondary to, and in the background of, chronic gastroesophageal reflux (Fig. 1-5).

• Patients with Barrett’s tend to have more severe esophageal motility disturbances (decreased lower esophageal sphincter pressure, ineffective peristalsis) and greater esophageal acid exposure on 24-hour pH monitoring than GERD patients without Barrett’s. • Intraesophageal bile (duodenogastroesophageal) reflux may also play a role in the pathogenesis. • Familial clustering of GERD and Barrett’s suggests a genetic predisposition for the disease. • The progression from metaplasia to carcinoma is associated with a number of changes in gene structure and expression.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

GERD, uncomplicated Erosive esophagitis Gastritis Peptic ulcer disease Angina Malignancy Stricture or Schatzki’s ring

WORKUP • Endoscopy with biopsy necessary for diagnosis. • Diagnosis requires the presence of intestinal metaplasia in columnar epithelium displaced proximal to the gastroesophageal junction (see Fig. 1-6). Longer segment Barrett’s is more readily diagnosed, but the length of the segment does not define the diagnosis. • Intestinal metaplasia of the gastric cardia is not considered Barrett’s and does not appear to convey the same risk of malignant transformation. • Imaging studies are nonspecific and insensitive for the diagnosis. • Screening for H. pylori infection in patients with GERD and Barrett’s esophagus is not recommended.

TREATMENT The primary therapeutic goal is to control GERD symptoms and maintain healed mucosa.

NONPHARMACOLOGIC THERAPY Same as treatment for GERD alone (lifestyle modifications, elevating the head of the bed, avoiding chocolate, tobacco, caffeine, mints, avoiding certain drugs [see Gastroesophageal reflux disease]); however, chronic acid suppression is often necessary to control symptoms and promote healing.

ACUTE GENERAL Rx • Proton pump inhibitors (PPIs) are most effective at relieving symptoms and healing mucosal injury. • Adequate control of GERD symptoms in patients with Barrett’s esophagus may or may not completely control intraesophageal acid exposure. Some studies have suggested that normalization of intraesophageal acid exposure may lead to either regression of Barrett’s or reduce the risk of dysplasia. Large-scale longitudinal studies that would support a general recommendation of aggressive pH control and monitoring have not been performed or reported. • If asymptomatic and incidentally found to have Barrett’s esophagus, medication use may be considered for the above-mentioned reasons.

CHRONIC Rx • Thermal ablation techniques, photodynamic therapy, and endoscopic mucosal resection have all been suggested as possible approaches in patients with Barrett’s and high-grade dysplasia, either in conjunction with aggressive surveillance, or as an alternate to surgery in poor operative candidates. All of these options have significant risks and run the risk of residual intestinal metaplasia. • Antireflux surgery may be considered for management of GERD and associated sequelae. Surgical resection is offered for multifocal high-grade dysplasia or carcinoma.

SCREENING

FIGURE 1-5 Endoscopic view of the distal esophagus from a patient with gastroesophageal reflux disease showing a tongue of Barrett’s mucosa (b) and a Schatzki’s ring (s) (arrow). (From Goldman L, Bennett JC [eds]: Cecil textbook of medicine, ed 21, Philadelphia, 2000, WB Saunders.)

• GERD highly prevalent in general population. • Only 4% to 10% of patients with reflux symptoms develop Barrett’s esophagus. • Patients with chronic GERD symptoms should be considered for a one-time endoscopy to exclude the presence of Barrett’s. Because many patients with Barrett’s are asymptomatic, some will be missed; however, general population screening is not currently recommended.

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Barrett’s Esophagus DISPOSITION

REFERRAL • For endoscopy with biopsy in patients with chronic GERD symptomatology who have not had previous endoscopy • For surveillance in those with a previous biopsy-proven diagnosis of Barrett’s esophagus • For those with high-grade dysplasia, biopsies should be confirmed by an expert pathologist; patients should be offered intensive surveillance or esophageal resection; ablative therapy may be considered either as part of a research protocol, or if not an operative candidate

SUGGESTED READINGS Bammer T et al: Rationale for surgical therapy of Barrett esophagus, Mayo Clin Proc 76:335, 2001. Cameron A: Management of Barrett’s esophagus, Mayo Clin Proc 73:5, 1998. Falk GW: Barret’s esophagus, Gastroenterology 122:1569, 2002. Falk GW: Current challenges in Barrett’s esophagus, Cleve Clin J Med 68:415, 2001. Hirota WK: Specialized intestinal metaplasia, dysplasia, and cancer of the esophagus: prevalence and clinical date, Gastroenterology 116:277, 1999. Morales TG, Sampliner RE: Barrett’s esophagus, Arch Intern Med 159:1411, 1999. Oatu-Lasear R, Fitzgerald RC, Triadafilopoulas G: Differentiation and proliferation in Barrett’s esophagus and the effects of acid suppression, Gastroenterology 117:327, 1999. Provenzale D, Schmitt C, Wong JB: Barrett’s esophagus: a new look at surveillance based on emerging estimates of cancer risk, Am J Gastroenterol 94:2043, 1999. Rajan E, Burgart LJ, Gostout CJ: Endoscopic and histologic diagnosis of Barrett esophagus, Mayo Clin Proc 76:217, 2001. Samplinear RE and the Practice Parameters Committee of the American College of Gastroenterology: Updated guidelines for the diagnosis, surveillance, and therapy of Barrett’s esophagus, Am J Gastroenterol 97:1888, 2002. Shaheen N et al: Gastroesophageal reflux, Barrett esophagus, and esophageal cancer: clinical applications, JAMA 287(15):1982, 2002. Sharma P: Short segment Barrett esophagus and specialized columnar mucosa at the gastroesophageal junction, Mayo Clin Proc 76:331, 2001. Spechler SJ, Barr B: Review article: screening and surveillance of Barrett’s esophagus: what is a cost-effective framework? Aliment Pharmacol Ther 19(Suppl 1):49, 2004. Spechler SJ: Barrett’s esophagus, N Engl J Med 346:836, 2002. Wang KK, Samplinear RE: Mucosal ablation therapy of Barrett esophagus, Mayo Clin Proc 76:433, 2001. Wijnhoven BPL et al: Molecular biology of Barrett’s adenocarcinoma, Ann Surg 233: 322, 2001. AUTHOR: HARLAN G. RICH, M.D.

FIGURE 1-6 Epithelial metaplasia (original magnification, x16). The esophageal mucosa consists of columnar epithelium (Barrett’s esophagus) intermixed with squamous epithelium. (Photomicrograph courtesy Frank Mitros, M.D., Department of Pathology, University of Iowa. From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

DISEASES AND DISORDERS

mation and extensive mucosal sampling. Consideration may be given to intensive surveillance every 3 months for patients with focal high-grade dysplasia. Patients with multifocal highgrade dysplasia or carcinoma should be considered for resection or ablation if not an operative candidate. • Patients should be treated aggressively for GERD before surveillance.

Section I

• Overall, 30 to 50 times increased risk of adenocarcinoma of the esophagus in patients with Barrett’s esophagus than in general population • This risk corresponds to 500 cancers per yr per 100,000 persons with Barrett’s esophagus • Specifics of frequency of monitoring are controversial, no prospective controlled studies to prove that surveillance increases life expectancy • ACG recommends that patients with Barrett’s undergo surveillance endoscopy and systematic 4-quadrant biopsy at intervals determined by the presence and grade of dysplasia. All mucosal abnormalities should be biopsied as well. Patients who have had two endoscopies showing no evidence of dysplasia should have follow-up every 3 years. Patients with low-grade dysplasia should have extensive mucosal sampling, and then follow-up every year. Patients with high-grade dysplasia should have expert confir-

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Basal Cell Carcinoma BASIC INFORMATION DEFINITION Basal cell carcinoma (BCC) is a malignant tumor of the skin arising from basal cells of the lower epidermis and adnexal structures. It may be classified as one of six types (nodular, superficial, pigmented, cystic, sclerosing or morpheaform, and nevoid). The most common type is nodular (21%); the least common is morpheaform (1%); a mixed pattern is present in approximately 40% of cases. Basal cell carcinoma advances by direct expansion and destroys normal tissue.

SYNONYMS BCC ICD-9CM CODES 179.9 Basal cell carcinoma, site unspecified 173.3 Basal cell carcinoma, face 173.4 Basal cell carcinoma, neck, scalp 173.5 Basal cell carcinoma, trunk 173.6 Basal cell carcinoma of the limb 173.7 Basal cell carcinoma, lower limb

EPIDEMIOLOGY & DEMOGRAPHICS • Most common cutaneous neoplasm in humans (400,000 cases/yr) • 85% appear on the head and neck region • Most common site: nose (30%) • Increased incidence with age 40 yr • Increased incidence in men • Risk factors: fair skin, increased sun exposure, use of tanning salons with ultraviolet A or B radiation, history of irradiation (e.g., Hodgkin’s disease), personal or family history of skin cancer, impaired immune system

PHYSICAL FINDINGS & CLINICAL PRESENTATION Variable with the histologic type: • Nodular: dome-shaped, painless lesion that may become multilobular and frequently ulcerates (rodent ulcer); prominent telangiectatic vessels are noted on the surface; border is translucent, elevated, pearly white (Fig. 1-7);

some nodular basal cell carcinomas may contain pigmentation, giving an appearance similar to a melanoma. • Superficial: circumscribed scaling black appearance with a thin raised pearly white border; a crust and erosions may be present; occurs most frequently on the trunk and extremities. • Morpheaform: flat or slightly raised yellowish or white appearance (similar to localized scleroderma); appearance similar to scars, surface has a waxy consistency.

• •

•

ETIOLOGY Sun exposure and use of tanning salons with equipment that emits ultraviolet A or B radiation

DIAGNOSIS

•

DIFFERENTIAL DIAGNOSIS • Keratoacanthoma • Melanoma (pigmented basal cell carcinoma) • Xeroderma pigmentosa • Basal cell nevus syndrome • Molluscum contagiosum • Sebaceous hyperplasia • Psoriasis

WORKUP Biopsy to confirm diagnosis

•

and tumors with poorly defined clinical margins. Electrodesiccation and curettage: useful for small (6 mm) nodular basal cell carcinomas. Cryosurgery with liquid nitrogen: useful in basal cell carcinomas of the superficial and nodular types with clearly definable margins; no clear advantages over the other forms of therapy; generally reserved for uncomplicated tumors. Radiation therapy: generally used for basal cell carcinomas in areas requiring preservation of normal surrounding tissues for cosmetic reasons (e.g., around lips); also useful in patients who cannot tolerate surgical procedures or for large lesions and surgical failures. Photodynamic therapy uses a skin cream (Metvix, known as methyl aminolevulinate cream) and concentrated light to activate the cream, which kills cancer cells. Is used in Europe and Australia but not yet FDAapproved in the U.S. Imiquimod (Aldara) 5% cream can be used for treatment of small, superficial BCCs of the trunk and extremities. Efficacy rate is approximately 80%. Its main advantage is lack of scarring, which must be weighed against higher cure rates with surgical intervention.

TREATMENT

CHRONIC Rx

NONPHARMACOLOGIC THERAPY

Periodic evaluation for at least 5 yr because of increased risk of recurrence of another basal cell carcinoma (40% risk within 5 yr of treatment)

Avoidance of excessive tanning, use of sunscreens to prevent damage from excessive sun exposure

ACUTE GENERAL Rx Variable with tumor size, location, and cell type: • Excision surgery: preferred method for large tumors with well-defined borders on the legs, cheeks, forehead, and trunk. • Mohs’ micrographic surgery: preferred for lesions in high-risk areas (e.g., nose, eyelid), very large primary tumors, recurrent basal cell carcinomas,

DISPOSITION • More than 90% of patients are cured. • A lesion is considered low risk if it is 1.5 cm in diameter, is nodular or cystic, is not in a difficult-to-treat area (H zone of face), and has not been previously treated. • Nodular and superficial basal cell carcinomas are the least aggressive. • Morpheaform lesions have the highest incidence of positive tumor margins (30%) and the greatest recurrence rate. AUTHOR: FRED F. FERRI, M.D.

FIGURE 1-7 Basal cell carcinoma. Note rolled translucent border and central ulceration in typical facial location. (From Noble J et al: Textbook of primary care medicine, ed 3, St Louis, 2001, Mosby.)

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Bell’s Palsy BASIC INFORMATION Bell’s palsy is an idiopathic, isolated, usually unilateral facial weakness in the distribution of the seventh cranial nerve (1% of the facial palsies are bilateral) Idiopathic facial paralysis ICD-9CM CODES 351.0 Bell’s palsy

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: 13-34 cases/100,000 persons RISK FACTORS: • Diabetes (present in 5%-10% of patients) • Travel to area endemic for Lyme disease

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Unilateral paralysis of the upper and lower facial muscles (asymmetric eye closure, brow, and smile). Upward rolling of eye on attempted eye closure (“Bell’s phenomenon”) • Ipsilateral loss of taste • Ipsilateral ear pain, usually 2-3 days before presentation • Increased or decreased unilateral eye tearing • Hyperacusis • Subjective ipsilateral facial numbness • In about 8% of cases, other cranial neuropathies may occur

ETIOLOGY • Most cases are idiopathic. • The cause is often viral (herpes simplex). • Herpes zoster can cause Bell’s palsy in association with herpetic blisters affecting the outer ear canal or the area behind the ear (Ramsay-Hunt syndrome). • Bell’s palsy can also be one of the manifestations of Lyme disease.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Neoplasms affecting the base of the skull or the parotid gland • Bacterial infectious process (meningitis, otitis media, osteomyelitis of the base of the skull) • Brainstem stroke • Multiple sclerosis

WORKUP Bell’s palsy is a clinical diagnosis. A focused history and neurologic examination will confirm the diagnosis.

LABORATORY TESTS • Fasting blood sugar to evaluate for diabetes • Consider CBC, VDRL, ESR, ACE in selected patients • Lyme titer in endemic areas

IMAGING STUDIES • Contrast-enhanced MRI to exclude neoplasms is indicated only in patients with atypical features or course. • Chest x-ray examination may be useful to exclude sarcoidosis or to rule out TB in selected patients before treating with steroids.

TREATMENT NONPHARMACOLOGIC THERAPY • Reassure patient that the disease is most likely a result of a virus attacking the nerve, not a stroke. It is also important to inform the patient that the prognosis is usually good. • Avoid corneal drying by applying skin tape to the upper lid to keep the palpebral fissure narrowed. Lacri-Lube ophthalmic ointment at night and artificial tears during the day are also useful to prevent excessive drying.

ACUTE GENERAL Rx • Although the benefits of corticosteroid therapy remain unproven, most practitioners use a brief course of prednisone therapy. Combination therapy with acyclovir and prednisone may possibly be effective in improving clinical recovery. • If used, prednisone therapy should be started within 24-48 hr of symptom onset. • Optimal steroid dose is unknown. Prednisone can be given as one 50-mg tablet qd for 7 days without tapering or can be started at 80 mg and tapered by 5 mg/day until finished. A Medrol dose-pack may also be given. A randomized controlled trial of patients treated with high dose IV steroids within 72 hr compared with placebo

CHRONIC Rx Patients should be monitored for evidence of corneal abrasion and ulceration or hemifacial spasm. Physical therapy including moist heat and massage may be beneficial.

DISPOSITION • 71% of patients should recover completely. Prognosis is improved for those with clinical improvement within 3 wk and with less severity of symptoms at onset. • Recovery begins within 3 wk in 85% of patients, with the remainder having some improvement within 3-6 mo. • Recurrence is experienced in 5% of Bell’s palsy cases.

REFERRAL • Persistent redness or irritation of the eye requires referral to an ophthalmologist. • Neurology referral is recommended if diagnosis is unclear or if the clinical course is atypical.

SUGGESTED READINGS Benatar M, Edlow JA: The spectrum of cranial neuropathy in patients with Bell’s palsy, Arch Intern Med 164:2283, 2004. Grogan PM: Practice parameter: steroids, acyclovir, and surgery for Bell’s palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology, Neurology 56(7): 830, 2001. Holland NJ, Weiner GM: Recent developments in Bell’s palsy, Br J Med 329:553, 2004. Jabor MA, Gianoli G: Management of Bell’s palsy, J La State Med Soc 148:279, 1996. Lagalla G et al: Influence of early high-dose steroid treatment on Bell’s palsy evolution, Neurol Sci 23:107, 2002. Mountain RE et al: The Edinburgh facial palsy clinic: a review of three years’ activity, J R Coll Surg Edinb 39:275, 1994. Peitersen E: The natural history of Bell’s palsy, Am J Otol 4:107, 1982. AUTHOR: RICHARD S. ISAACSON, M.D.

DISEASES AND DISORDERS

SYNONYMS

found a significant improvement in recovery rate and time to return to work but no statistical difference in final outcome. • There is preliminary evidence for the effectiveness of methylcobalamin (active form of vitamin B12) and hyperbaric oxygen, but these have not yet received widespread acceptance. • Botulinum toxin may be helpful for the treatment of synkinesis and hemifacial spasm, two of the late sequelae of Bell’s palsy. • Treat Lyme disease if present

Section I

DEFINITION

• Sarcoidosis • Head trauma with fracture of temporal bone • Other: Guillain-Barré, carcinomatous or leukemic meningitis, leprosy, Melkersson-Rosenthal syndrome

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Benign Prostatic Hyperplasia (BPH) PHYSICAL FINDINGS & CLINICAL PRESENTATION • Irritative symptoms are usually the first

BASIC INFORMATION DEFINITION

Multifactorial; a functioning testicle is necessary for development of BPH (as evidenced by the absence of BPH in males who were castrated before puberty).

to manifest. The hallmark is urgency; the patient has progressively less time between the first sensation of bladder fullness and an irresistible urge to void. “When I have to go, I need to go real fast.” These symptoms progress in association with reduced urinary flow, especially in the morning. In time, obstructive symptoms take center stage with difficulty initiating flow (hesitancy), dribbling during micturition, incomplete emptying of the bladder resulting in double-voiding (need to urinate again a few minutes after voiding), and postvoid dribbling or incontinence. Eventually, obstructive symptoms can lead to urinary retention. • Digital rectal examination (DRE) reveals enlargement of the prostate. • Focal enlargement may be indicative of malignancy. • Correlation between size of prostate and symptoms is poor (BPH may be asymptomatic if it does not encroach on the urethral lumen).

Benign prostatic hyperplasia is the benign growth of the prostate, generally originating in the periureteral and transition zones, with subsequent irritative and obstructive voiding symptoms.

SYNONYMS BPH Prostatic hypertrophy ICD-9CM CODES 600 Benign prostatic hyperplasia

EPIDEMIOLOGY & DEMOGRAPHICS • 80% of men have evidence of benign prostatic hypertrophy by age 80 yr. • Medical and surgical intervention for problems caused by BPH is required in 20% of males by age 75 yr. • Transurethral resection of the prostate (TURP) is the tenth most common operative procedure (400,000/yr in U.S.). • 10% to 30% of men with BPH have occult prostate cancer.

TABLE 1-1

ETIOLOGY

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Prostatitis Prostate cancer Strictures (urethral) Medication interfering with the muscle fibers in the prostate and also with bladder function

WORKUP Symptom assessment as described previously (may use American Urological Association [AUA] or Symptom Index for BPH [Table 1-1]), laboratory tests, and imaging studies

LABORATORY TESTS • Prostate-specific antigen (PSA) to screen for prostate cancer: protease secreted by epithelial cells of the prostate; elevated in 30% to 50% of patients with BPH; also increases with

International Prostate Symptom Score (I-PSS)

SCORE

Symptom Incomplete emptying: Over the past month, how often have you had a sensation of not emptying your bladder completely after you finished urinating? Frequency: Over the past month, how often have you had to urinate again 2 hr after you finished urinating? Intermittency: Over the past month, how often have you found you stopped and started again several times when you urinated? Urgency: Over the past month, how often have you found it difficult to postpone urination? Weak stream: Over the past month, how often have you had a weak urinary stream? Straining: Over the past month, how often have you had to push or strain to begin urination? Nocturia: Over the past month, how many times did you most typically get up to urinate from the time you went to bed at night until the time you got up in the morning? Total I-PSS score 

Not at All

Less than 1 Time in 5

Less than Half the Time

About Half the Time

More than Half the Time

Almost Always

Total Score

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

0

1

2

3

4

5

None

1 Time

2 Times

3 Times

4 Times

5 or More Times

0

1

2

3

4

5

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Benign Prostatic Hyperplasia (BPH)

• Transrectal ultrasound may be indicated in patients with palpable nodules or significant elevation of PSA. It is also useful to estimate prostate size. • Uroflowmetry may be used to determine relative impact of obstruction on urine flow (not routine). • Pressure flow studies, although invasive, are useful in occasional patients for whom a distinction between prostatic obstruction and impaired detrusor contractility may affect the choice of therapy. • Postvoid residual (PVR) urine measurement may identify patients who should not receive anticholinergic drugs (PVR 100 ml). • Cystoscopy is an option during later evaluation if invasive treatment is being planned.

NONPHARMACOLOGIC THERAPY • Avoidance of caffeine or any other foods that may exacerbate symptoms. • Avoidance of medications that may exacerbate symptoms (e.g., most cold and allergy remedies). Indeed, anticholinergic drugs (e.g., antihistamines) decrease detrusor contractility, and sympathomimetics (e.g., decongestants) increase bladder outlet pressure. They are relatively contraindicated in anyone with a PVR 100 cc. • The dietary supplement saw palmetto is effective in relieving BPH symptoms in some patients with mild obstruction.

GENERAL Rx

limited to patients whose estimated resection tissue weight would be 30 g or less. TUIP can be performed in an ambulatory setting or during a 1-day hospitalization. Open prostatectomy is typically performed on patients with very large prostates. Surgery may result in significant complications (e.g., incontinence, infection). • Laser therapy for BPH is a less invasive alternative to TURP; however, TURP is moderately more effective than laser therapy in relieving symptoms of BPH. • Balloon dilation of the prostatic urethra is less effective than surgery for relieving symptoms but is associated with fewer complications. It is a reasonable treatment option for patients with smaller prostates and no middle lobe enlargement.

• Asymptomatic patients with prostate enlargement caused by BPH generally do not require treatment. Patients with mild to moderate symptoms are candidates for pharmacologic treatment (see as follows).

CHRONIC Rx

MEDICAL TREATMENT

DISPOSITION

• Avoid medications and foods that exacerbate symptoms. • Symptomatic improvement occurs in 70% of patients with proper treatment.

• -1 blockers (e.g., tamsulosin, alfuzosin, doxazosin, prazosin, and terazosin) relax smooth muscle of the bladder neck and prostate and can increase urinary flow rate. They have no effect on the size of the prostate. -1 blockers are useful in symptomatic patients to relieve symptoms of obstruction by causing relaxation of smooth muscle tone in the prostatic capsule and urethra and bladder neck. Tamsulosin and alfuzosin are more specific to bladder -1 receptors and do not cause much hypotension. Prazosin, doxazosin, and terazosin are nonspecific -1 blockers and are used to treat hypertension; they can cause (postural) hypotension. -1 blockers are the most effective medical pharmacologic therapy for BPH-associated lower urinary tract symptoms. • Hormonal manipulation with finasteride (Proscar) 5 mg qd or dutasteride (Avodart) 0.5 mg qd. Both are 5 -reductase inhibitors that block conversion of testosterone to dihydrotestosterone; they can reduce the size of the prostate. Treatment requires 6 mo or more for maximal effect.

With appropriate therapy, symptoms improve or stabilize in 70% of patients with BPH.

SURGICAL TREATMENT

SUGGESTED READINGS

• TURP is the most commonly used surgical procedure for BPH. Transurethral incision of the prostate (TUIP), a procedure almost equivalent in efficacy, is

Dull P et al: Managing benign prostatic hyperplasia, Am Fam Physician 66:77, 2002.

REFERRAL Urology referral for patients with severe or intolerable symptoms and for any patient suspected of having prostate cancer (10% to 30% of men with BPH)

PEARLS & CONSIDERATIONS COMMENTS Emerging technologies for treating BPH include lasers, coils, stents, thermal therapy, and hyperthermia. Laser prostatectomy appears promising; however, longterm effectiveness has not yet been demonstrated. • The increase in the use of pharmacologic management has resulted in over 30% reduction in the total number of transurethral resections of the prostate. • Caution: 5 -reductase inhibitors cut PSA levels in half. Therefore, the PSA should be doubled to interpret the result in a patient using such treatment.

AUTHORS: FRED F. FERRI, M.D., and TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

IMAGING STUDIES

TREATMENT

Section I

age independent of prostate size. Therefore, the PSA test does not discriminate well between patients with BPH and those with prostate cancer, particularly if the cancers are pathologically localized and curable. Nonetheless, testing for PSA increases detection rate for prostate cancer and tends to detect cancer at an earlier stage. PSA testing and digital rectal examination should be offered to any asymptomatic man older than 50 years of age with a life expectancy of 10 years. PSA testing can also be offered at an earlier age in men at higher risk of prostatic cancer (e.g., first-degree relatives with prostate cancer; black men). • Measurement of “free” PSA is useful to assess the probability of prostate cancer in patients with normal digital rectal examination and total PSA between 4 and 10 ng/ml. In these patients the global risk of prostate cancer is 25%; however, if the free PSA is 25%, the risk of prostate cancer decreases to 8%, whereas if the free PSA is 10%, the risk of cancer increases to 56%. Another approach is to measure “PSA velocity.” Repeat PSA measurements are obtained every 3 months to assess the rate of increase. PSA should increase by less than 0.85 ng/ml per year and should take at least 4 years to double. • Urinalysis, urine C&S to rule out infection (if suspected). • BUN and creatinine to rule out postrenal insufficiency.

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Bladder Cancer BASIC INFORMATION DEFINITION Bladder cancer is a heterogeneous spectrum of neoplasms ranging from non–life-threatening, low-grade, superficial papillary lesions to high-grade invasive tumors, which often have metastasized at the time of presentation. It is a field change disease in which the entire urothelium from the renal pelvis to the urethra may be susceptible to malignant transformation. Types: Transitional cell carcinoma (TCCa), squamous cell carcinoma, and adenocarcinoma. ICD-9CM CODES Primary: 188.9 Secondary: 198.1 CIS: 233.7 Benign: 223.3 Uncertain behavior: 236.7 Unspecified: 239.4

EPIDEMIOLOGY & DEMOGRAPHICS Each year approximately 54,000 new cases are diagnosed and more than 12,000 deaths are attributed to bladder cancer. Until 1990, the incidence of bladder cancer in the U.S. was rising. Since 1990, the incidence of bladder cancer is decreasing at a rate of 0.8% per year (1.2% among men and 0.4% among women). PREDOMINANT SEX: In males, it is the fourth most common cancer; it accounts for 10% of all cancers. In females, it is the eighth most common cancer; it accounts for 4% of all cancers. RISK: The lifetime risk of developing bladder cancer is 2.8% in white males, 0.9% in black males, 1% in white females, and 0.6% in black females. Smoking: • Users of “black” tobacco in place of “blond” tobacco have a twofold to threefold increase in developing bladder cancer. • Smoking risk is based on consumption: With a twofold to threefold increase for subjects smoking at least 10 cigarettes per day The risk increases again when the daily consumption rises above 40-60 cigarettes per day • Smokers of low-tar and nicotine cigarettes have a lower risk when compared with higher tar and nicotine cigarettes. • Unfiltered cigarettes have a 50% increased risk of bladder cancer compared with those who smoke filtered cigarettes. • Pipe smokers have a lower risk of bladder cancer compared with cigarette smokers.

• Cigar smoking, snuff, and chewing tobacco, although implicated in nonurologic cancers, are not believed to influence bladder cancer risk. Diet: • Diets rich in beef, pork, and animal fat consumption increase risk of bladder cancer. • There is no indication that consumption of nonbeer alcoholic drinks contributes to bladder cancer development. • Beer consumption has been linked to bladder cancer development as a result of the presence of nitrosamines in the beer. Similarly, these nitrosamines have been implicated in the development of rectal cancer. • Drinking coffee is not believed to contribute to bladder cancer risk. There is additional evidence that coffee consumption is protective for colorectal cancers, possibly by diminishing fecal transit time. PEAK INCIDENCE: Incidence increases with age, high 60 yr, uncommon 40 yr. GENETICS: It is thought to be multifactorial in etiology, involving both genetic and environmental interactions. Overall, it is estimated that approximately 20% to 25% of the male population in the U.S. with bladder cancer has the disease as a result of occupational exposure. DISTRIBUTION: In North America, transitional cell carcinomas comprise 93%, squamous cell carcinomas comprise 6%, and adenocarcinomas account for 1% of bladder cancers. PATHOGENESIS: Two pathways exist for bladder cancer (TCCa): 1. Papillary superficial disease occasionally leading to invasive cancer (75%) 2. Carcinoma-in-situ (CIS) and solid invasive cancer with high risk of disease progression (25%) Two distinct forms of “Superficial Cancer” exist: Ta Papillary low-grade tumor. High rate of recurrence. Disease progression occurs 5% T1 Higher-grade papillary tumors that infiltrate the lamina propria. Often associated with flat CIS that may involve the urothelium diffusely. Disease progression occurs between 30% and 50% Subdivided into: T1a Penetration of tumor up to the muscularis mucosae. Disease progression 5.3% T1b Penetration of tumor through the muscularis mucosae. Disease progression 53% Flat CIS: Entirely different and separate pathway of cancer development whose mechanism is manifested by dyspla-

sia, which leads to the occurrence of poorly differentiated malignant cells that replace or undermine the normal urothelium and extend along the plane of the bladder wall. It penetrates the basement membrane and lamina propria in 20% to 30% of the cases and is associated with the development of solid tumor growth. A defect in chromosome 17p53 occurs in 50% of the cases. At presentation, 72% of cancers are localized to the bladder, 20% of the cancers extend to the regional lymph nodes, and 3% present with distant metastases. 80% of superficial TCCa recur with up to 30% progressing to a higher stage or grade. Younger patients most commonly develop low-grade papillary noninvasive TCCa and are less likely to have recurrences when compared with older patients with similar lesions. Involvement of the upper tracts with tumor occurs in 25% to 50% of the cases. STAGING (BASED ON THE TNM SYSTEM): T0 No tumor in specimen Tis CIS Ta Papillary TCCa noninvasive T1 Papillary TCCa into lamina propria T2 TCCa invasive of superficial ms T3a Invasive of deep ms T3b Invasive of perivesical fat T4a Invasive of adjacent pelvic organ T4b Invasive of pelvic wall with fixation Invasive of nodal status: N0 No nodal involvement N1-3 Pelvic nodes N4 Nodes above bifurcation Nx Unknown Invasive of metastatic status: M0 No distant metastases M1 Distant metastases Mx Unknown MOLECULAR EPIDEMIOLOGY: TCCa is usually a field change disease with tumors arising at different times and sites in the urothelium, suggesting a polyclonal etiology of bladder cancer. Bladder cancers have been associated with abnormalities on chromosomes 1, 4, 11, 5, 7, 3, 9, 21, 18, 13, 8; with alterations in suppressor genes P53, retinoblastoma gene, and P16; and with alterations in oncogenes H-ras and epidermal growth factor receptor.

PHYSICAL FINDINGS • Gross painless hematuria • Microhematuria • Frequency, urgency, occasional dysuria With locally invasive to distant metastatic disease, the presentation can include: • Abdominal pain • Flank pain

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Bladder Cancer Lymphedema Renal failure Anorexia Bone pain

bladder cancer tumor markers. No marker has general, widespread acceptance because the results are affected by the presence of stents, recent urologic manipulation, stones, infection, bowel interposition, and prostatitis creating false-positive results.

ETIOLOGY

DIAGNOSIS • History and physical examination • Urinalysis • Cystoscopy with bladder barbotage and biopsy • Transurethral resection of bladder tumor(s) • There is insufficient evidence to determine whether a decrease in mortality from bladder cancer occurs with hematuria testing, urinary cytology, or a variety of other tests on exfoliated urinary cells or other substances. • In addition to urinary cytologies and bladder barbotage, BTA, NMP22, and Fibrin Degradation Products (FDP) have been approved by the FDA as

DIFFERENTIAL DIAGNOSIS • • • • • •

Urinary tract infection Frequency-urgency syndrome Interstitial cystitis Stone disease Endometriosis Neurogenic bladder

LABORATORY TESTS RADIOLOGIC TESTS: • IVP, renal ultrasound, retrograde pyelography, CT scan, and MRI. • One or a combination of studies can be used. In the absence of skeletal symptoms, bone scan is not recommended.

• • • • •

Contraindications:

• • • • • • • • •

TREATMENT NONPHARMACOLOGIC THERAPY • Initially, transurethral resection of bladder tumor (TURBT) • Loop biopsy of the prostatic urethra if high-grade TCCa is suspected • If superficial disease, follow-up protocol with repeat TURBT and/or the use of intravesical agents is recommended • For advanced bladder cancer, radical cystectomy with urethrectomy (unless orthotopic diversion is planned) and either ileal loop conduit or orthotopic diversion BLADDER PRESERVATION APPROACHES: Following cystectomy for muscle invasive disease, 50% or more of the patients will develop metastases. Most patients develop metastases at distant sites, a third relapse locally. Bladder preservation management is offered in those individuals who refuse surgery or who might not be suitable radical cystectomy patients. Bladder-sparing protocols include extensive TURBT or partial cystectomy with external beam or interstitial radiotherapy and systemic chemotherapy. Radiotherapy as a single treatment modality is not effective. The best predictor of successful bladder preservation is a complete response following the combination of initial TURBT and two cycles of CMV (cisplatin, methotrexate, vinblastine) chemotherapy seen with stages T2-T3a. INDICATIONS FOR PARTIAL CYSTECTOMY: • Tumor within a bladder diverticulum • Solitary, primary, and muscle-invasive or high-grade lesion of a region of the

•

Multiple tumors CIS Cellular atypia on biopsy Prostatic invasion Invasion of the trigone Inability to achieve adequate surgical margins Prior radiotherapy Inability to maintain adequate bladder volume after resection Evidence of extravesical tumor extension Poor surgical risk

ACUTE GENERAL Rx INDICATIONS FOR INTRAVESICAL CHEMOTHERAPY: • High-grade tumor • Tumor size 5 cm • Tumor multiplicity • Presence of CIS • Positive urinary cytologies following a resection • Incomplete tumor resection Intravesical agents: thiotepa, Adriamycin, mitomycin C, AD-32, BCG, interferon, bropirimine, interleukin-2, and keyholelimpet hemocyanin. Photodynamic therapy with hematoporphyrin derivatives has also been used. INDICATIONS FOR CYSTECTOMY: • Large tumors not amenable to complete TURBT • High-grade tumor • Multiple tumors with frequent recurrences • Diffuse CIS not responsive to intravesical chemotherapy • Prostatic urethra involvement • Irritative bladder symptoms with upper tract deterioration • Muscle-invasive disease • Disease outside of the bladder SYSTEMIC CHEMOTHERAPY: Used as neoadjuvant and adjuvant therapy for systemic disease. The most effective agents are cisplatin, methotrexate, vinblastine, Adriamycin (MVAC). Other agents include mitoxantrone, vincristine, etoposide (VP16), 5FU, ifosfamide, Taxol, gemcitabine, piritrexim, and gallium nitrate. Chemotherapy in combination can provide palliation and modest survival benefit.

DISEASES AND DISORDERS

Bladder cancer is a potentially preventable disease associated with specific etiologic factors: • Cigarette smoking is associated with 25% to 65% of the cases. The risk of developing a TCCa is 2 to 4 times higher in smokers than in nonsmokers, and that risk persists for many years, being equal to nonsmokers only after 12 to 15 yr of smoking abstinence. Smoking tobacco is associated with tumors that are characterized by higher histologic grade, increased tumor stage, increase in the numbers of tumors present, and increased tumor size. • Occupational exposures: dye workers, textile workers, tire and rubber workers, petroleum workers • Chemical exposure: O-toluidine, 2naphthylamine, benzidine, 4-aminobiphenyl, and nitrosamines • Exposure to HPV type 16 Squamous carcinomas are associated with: • Schistosomiasis • Urinary calculi • Indwelling catheters • Bladder diverticula Miscellaneous causes: • Phenacetin abuse • Cyclophosphamide • Pelvic irradiation • Tuberculosis Adenocarcinomas are associated with: • Exstrophy • Endometriosis • Neurogenic bladder • Urachal abnormalities • As a secondary site for distant metastases from other organs (i.e., colon cancer)

•

bladder that allows complete excision with adequate surgical margins Inability to adequately resect tumor by TURBT alone because of size or location Tumor overlying a ureteral orifice requiring ureteral reimplantation Biopsy of a radiation-induced ulceration Palliation of severe local symptoms Patient refusal of urinary diversion Poor-risk patient who is not a diversion candidate

Section I

• • • •

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Bladder Cancer RADIOTHERAPY: Conflicting reports suggest that superficial bladder cancer is more sensitive to radiotherapy. Squamous changes within the tumor and secretion of human chorionic gonadotropin by the lesion are associated with poor response to radiotherapy. Only 20% to 30% of patients with invasive bladder cancer can be cured by external beam radiation therapy alone. It is used in combination with surgery or with systemic agents to treat bladder cancer primarily in those patients who are not surgical candidates or who refuse surgery.

• Upper tract studies are based on the risk of upper tract tumor development, generally every 2 to 5 yr. FOLLOW-UP RECOMMENDATIONS FOR ADVANCED DISEASE:

CHRONIC Rx

• Neobladder endoscopy and IVP yearly. • CT scan of abdomen and pelvis every 6 mo for 2 yr in addition to chest x-ray examination, liver function tests, and serum creatinine. • Loopogram every 6 mo for 2 yr, then yearly.

FOLLOW-UP RECOMMENDATIONS FOR SUPERFICIAL BLADDER CANCER: • Cystoscopy, bladder barbotage, and bimanual examination every 3 mo for 2 yr, then every 6 mo for 2 yr, and annually thereafter.

Bladder Preservation:

• Cystoscopy, barbotage, bimanual examination, biopsy (when indicated), every 3 mo for 2 yr, then every 6 mo for 2 yr, yearly thereafter. • CT scan of abdomen and pelvis every 6 mo for 2 yr in addition to chest x-ray examination, liver function testing, and serum creatinine. Cystectomy with Ileal Loop/Orthotopic Bladder:

American Urological Association Guideline Recommendations BOX 1-2

1. Undiagnosed bladder tumor: obtain a histologic diagnosis of the tumor: Transurethral resection of the tumor is the most common method. 2. Stage Ta or T1 cancer: Complete surgical eradication of all visible tumors. The lesion can be treated with electrocautery resection, fulguration, or laser ablation. Adjuvant intravesical therapy is recommended for patients with carcinoma-in-situ, T1, or high-grade Ta tumors. The agent recommended is BCG or mitomycin C. Cystectomy is an option for this set of tumors because of risk of progression to muscle-invasive disease even after intravesical chemotherapy. An increased risk of disease progression is associated with large tumor, high-grade tumor, location of the tumor in a site that is poorly accessible to complete resection, diffuse disease, infiltration of lymphatic or vascular spaces, and prostatic urethral involvement. 3. Carcinoma-in-situ or high-grade T1 cancer and prior intravesical chemotherapy: Cystectomy is the recommendation based on the panel’s expert opinion rather than evidence from outcomes data. The data show a substantial risk of progression to muscle-invasive cancer in patients with diffuse carcinoma-in-situ and highgrade T1 tumors. The response to intravesical chemotherapy in terms of altering this disease progression is unknown, and as a result of this, cystectomy is an option for the afflicted patient. American Urological Association, Guideline Division, 1120 North Charles Street, Baltimore, MD 21201.

PEARLS & CONSIDERATIONS COMMENTS • The most useful prognostic parameters for bladder tumor recurrence and subsequent cancer progression are tumor grade, depth of tumor penetration, multifocal tumors, frequency of recurrence, tumor size, CIS, lymphatic invasion, papillary or solid tumor configuration. • Box 1-2 describes the American Urological Association Guideline Recommendations for bladder cancer.

SUGGESTED READINGS Lamm DL et al: Megadose vitamins in bladder cancer: a double-blind clinical trial, J Urol 151:21, 1994. Messing EM: In Walsh PC et al. (eds): Campbell’s urology, ed 8, Philadelphia, 2002, WB Saunders. AUTHOR: PHILIP J. ALIOTTA, M.D., M.S.H.A.

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Breast Cancer BASIC INFORMATION The term breast cancer refers to invasive carcinoma of the breast, whether ductal or lobular. Carcinoma of the breast ICD-9CM CODES 174.9 Malignant neoplasm female breast

EPIDEMIOLOGY & DEMOGRAPHICS • Nearly exclusively the disease of women, with only 1% of breast cancers in males • Steady increase in its incidence in the U.S., with 205,000 new patients annually • Annual mortality of 40,000 • Risk steadily increases with age • Genetically defined group of women with BRCA-1 or BRCA-2 identified to carry lifetime risk as high as 85%

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Increasing number of small breast cancers found by mammograms • Patients usually completely free of physical findings • Palpable tumors possibly as small as 1 cm or even smaller • Size of the mass and its location measured and documented

ETIOLOGY • Precise mechanism of carcinogenesis not understood • Possibly interaction of ovarian estrogen, nonovarian estrogen, estrogens of exogenous origin with breast tissue of varied carcinogenic susceptibility to develop cancer • Other known or suspected variables: childbearing, breast-feeding practice, diet, physical activities, body mass, alcohol intake • Have identified families with known high risk

DIAGNOSIS DIFFERENTIAL DIAGNOSIS The following nonmalignant breast lesions can simulate breast cancer on both physical and mammogram examinations: 1. Fibrocystic changes 2. Fibroadenoma 3. Hamartoma 4. Mastitis 5. Hematoma 6. Duct ectasia

Mammograms: 30% to 50% of breast cancers detected by screening mammograms only as a spiculated mass, a mass with or without microcalcifications, or a cluster of microcalcifications (Fig. 1-8)

WORKUP • Physical examination: 1. Mass detected by patient or medical professional: workup required 2. Negative mammogram: breast cancer not ruled out 3. Sonogram: to demonstrate mass to be cyst (not common in elderly); usually eliminating need for further workup • To establish diagnosis: 1. Positive aspiration cytology on a clinically and mammographically malignant mass—highly accurate but still requires open biopsy confirmation 2. Stereotactic core needle biopsy diagnosis: reliable with invasive carcinoma identified, but negative or equivocal results require careful evaluation 3. Atypical hyperplasia or in situ carcinoma found by core needle biopsy: open surgical biopsy confirmation still required 4. Excisional or incisional biopsy: establishes diagnosis • Note: Do not rely on negative mammogram or negative aspiration cytology to exclude malignancy. Make appropriate referral. Obtain imaging studies such as bone scan, chest x-ray examination, CT scan of abdomen, or CT scan of liver.

TREATMENT NONPHARMACOLOGIC THERAPY

A

B

• Early breast cancer: primarily surgical or surgical and radiotherapeutic. • Choice in 60% to 70% of women between modified mastectomy and breast-conserving treatment, which consists of lumpectomy, axillary staging with sentinel node biopsy or axillary dissection, and breast irradiation. • In elderly women lumpectomy without radiation therapy constitutes adequate primary breast treatment in many cases.

ACUTE GENERAL Rx

FIGURE 1-8 A, Right mediolateral and, B, spot magnification view from routine screening mammography demonstrates a small, ill-defined mass with minimal spiculation. This was nonpalpable, and biopsy demonstrated infiltrating ductal carcinoma. (From Specht N [ed]: Practical guide to diagnostic imaging, St Louis, 1998, Mosby.)

• May require adjuvant chemotherapy or endocrine therapy depending on receptor status of cancer cells for estrogen and progesterone • Evaluation and treatment by medical oncologist

DISEASES AND DISORDERS

SYNONYMS

IMAGING STUDIES Section I

DEFINITION

• Skin or nipple retraction and skin edema/erythema/ulcer/satellite nodule • Nodal enlargement in axilla and supraclavicular areas • Advanced disease: clinical signs of pleural effusion or hepatomegaly • Rare instances: clear, serous, bloody discharge, or eczematous dermatitis of nipple could be Paget’s disease of the breast

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Breast Cancer CHRONIC Rx Follow-up required after proper treatment of primary breast cancer includes: 1. Periodic clinical evaluations 2. Annual mammograms 3. Other tests as indicated 4. Patient instruction in monthly breast self-examination technique

DISPOSITION • Prognosis after curative therapy: depends on size of tumor, extent of nodal metastasis, and pathologic grade of tumor 1. Patient with 1-cm tumor with no axillary node metastasis: 10-yr disease-free survival rate of 90% 2. Patient with 3-cm tumor with metastasis in four nodes: 10-yr disease-free survival rate of 15% if no systemic adjuvant therapy given 3. Outlook for most patients between these extremes • Systemic adjuvant therapy: improves prognosis significantly

REFERRAL Referral is necessary as soon as breast cancer is even remotely suspected.

PEARLS & CONSIDERATIONS SCREENING • Breast self-exam monthly not proven effective but recommended nonetheless. • Breast exam by physician yearly. • Screening mammography yearly beginning at age 40, with no specific upper age limit. So long as a woman is a candidate for lumpectomy, she is also a candidate for screening.

Ductal carcinoma in situ (DCIS, intraductal carcinoma): 1. “New” disease mostly found by mammogram as cluster of microcalcification or density 2. Less often, presents as palpable mass or nipple discharge 3. Before mammogram screening, DCIS accounted for 1% of all breast cancers 4. Now, 15% to 20% or even higher proportion present with DCIS 5. Formerly treated with mastectomy, now lumpectomy 6. Cure rates 98% to 99% 7. No axillary dissection required 8. With radiation, breast recurrences reduced 9. Mastectomy possibly required with extensive or high-grade DCIS 10. Systemic adjuvant treatment is not indicated Inflammatory carcinoma: 1. Rare but rapidly progressive and often lethal form of breast cancer 2. Presents as erythematous and edematous breast resembling mastitis 3. Biopsy required, including skin 4. Treatment with combination chemotherapy followed by surgery and radiation therapy 5. Prognosis once dismal, now 5-yr disease-free survival in 50% of patients

COMMENTS • Patient education material can be obtained from the following: 1. SHARE: Self-Help for Women with Breast Cancer, 19 W 44th Street, No. 415, New York, NY 100365902. 2. Y-ME National Organization of Breast Cancer Information and Support, 18220 Harwood Avenue, Homewood, IL 80430.

• Breast radiologic evaluation, evaluation of nipple discharge, and evaluation of palpable mass are described in Section III.

SUGGESTED READINGS The ATAC Trialists’ Group: Anastrozole alone or in combination with tamoxifen versus tamoxifen alone for adjuvant treatment of postmenopausal women with early breast cancer: first results of the ATAC randomized trial, Lancet 359:2131, 2002. Boyd NF et al: Heritability of mammographic density, a risk factor for breast cancer, N Engl J Med 347:886, 2002. Hughes K et al: Post lumpectomy radiation not necessary for all elderly women, N Engl J Med 351:971-977, 2004. Hellekson KL: NIH statement on adjuvant therapy for breast cancer, Am Fam Physician 63:1857, 2001. Humphrey LL et al: Breast cancer screening: a summary of the evidence for the U.S. Preventive Services Task Force, Ann Intern Med 137:347, 2002. Marchbanks PA et al: Oral contraceptives and the risk of breast cancer, N Engl J Med 346:2025, 2002. Miller AB et al: The Canadian National Breast Screening Study—1: breast cancer mortality after 11 to 16 years of follow-up, Ann Intern Med 137:305, 2002. Pruthi S: Detection and evaluation of palpable breast mass, Mayo Clin Proc 76:641, 2001. Rebbeck TR et al: Prophylactic oophorectomy the risk of ovarian and breast cancer in carriers of BRCA1 or BRCA2 mutations, N Engl J Med 346:1616, 2002. U.S. Preventive Services Task Force: Chemoprevention of breast cancer. Recommendations and rationale, Ann Intern Med 137:56, 2002. AUTHORS: TAKUMA NEMOTO, M.D., with revisions by TOM J. WACHTEL, M.D.

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Bursitis CHRONIC Rx

DEFINITION

DIFFERENTIAL DIAGNOSIS

Bursitis is an inflammation of a bursa and is usually aseptic. A bursa is a closed sac lined with a synovial-like membrane that sometimes contains fluid that is found or that develops in an area subject to pressure or friction.

• Degenerative joint disease • Tendinitis (sometimes occurs in conjunction with bursitis) • Cellulitis (if bursitis is septic) • Infectious arthritis

SYNONYMS

Aspiration with Gram stain and C&S

• Aspiration if excessive fluid volume present, followed by application of compression dressing to prevent fluid reaccumulation (repeat aspiration may be required) • Steroid injection into bursa (1 ml of triamcinolone, 40 mg, mixed with 1 to 3 cc of Xylocaine depending on size of bursa) • NSAIDs

IMAGING STUDIES

DISPOSITION

• Plain radiography to rule out other potential or coexisting bone or joint problems (Fig. 1-9) • MRI

• Many bursal sacs “dry up” eventually. • Nonsurgical treatment is effective in most cases.

TREATMENT

For orthopedic consultation to assist in treatment of sepsis or for excision of chronic enlarged bursa when indicated

Housemaid’s knee (prepatellar bursitis) Weaver’s bottom (ischial gluteal bursitis) Baker’s cyst (gastrocnemius-semimembranosus bursa) ICD-9CM CODES 726.19 Subacromial bursitis 726.33 Olecranon bursitis 726.5 Ischiogluteal bursitis (hip) 726.5 Iliopsoas bursitis (hip) 726.61 Anserine bursitis 726.5 Trochanteric bursitis 726.65 Prepatellar bursitis 727.51 Baker’s cyst 726.79 Retrocalcaneal bursitis

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Swelling, especially if bursa is superficial (olecranon, prepatellar) • Local tenderness with pain on pressure against bursa • Pain with joint movement • Referred pain • Palpable occasional fibrocartilaginous bodies (most common in olecranon and prepatellar bursae)

ETIOLOGY • • • • •

Acute trauma Repetitive trauma Sepsis Crystalline deposit disease Rheumatoid arthritis

NONPHARMACOLOGIC THERAPY • If chronic, elimination of cause of pressure or irritation • Use of relief pads, avoidance of direct pressure • Rest • Elevation • Ice for acute trauma

ACUTE GENERAL Rx • Septic: 1. Appropriate antibiotic coverage and drainage 2. Aspiration of purulent fluid with a large-bore needle (if there is no rapid clinical response, incision and drainage are indicated) • Nonseptic: 1. Aspiration of blood from acute trauma 2. Application of compression dressing

REFERRAL

PEARLS & CONSIDERATIONS COMMENTS • Injection of trochanteric bursa may require spinal needle in large patient. • Sterile bursae should not be incised and drained because a chronic draining sinus tract may develop. • Involvement of the iliopsoas bursa may cause groin pain, although the diagnosis is difficult to make because of the inaccessibility of the area to direct examination. (This also makes steroid injection impossible even if the diagnosis could be established.)

SUGGESTED READINGS Floemer F, Morrison WB et al: MRI characteristics of olecranon bursitis, Am J Roentgenol 183:29, 2004. Sofka CM, Adler RS: Sonography of cubital bursitis, Am J Roengenal 183:51, 2004. Tortolani PJ, Carbone JJ, Quartaro LG: Greater trochantoric pain syndrome in patients referred to orthopedic spine specialists, Spine 2:251, 2002. Van Mieghem IM, Boets A et al: Ischiogluteal bursitis: an uncommon type of bursitis, Skeletal Radiol 33:413, 2004. Webner D, Drezner JA: Lesser trochanteric bursitis: a rare cause of anterior hip pain, Clin J Sport Med 14:242, 2004. AUTHOR: LONNIE R. MERCIER, M.D.

FIGURE 1-9 A, Bursae around the knee. B, Markedly swollen prepatellar bursa. (From Scudieri G [ed]: Sports medicine principles of primary care, St Louis, 1997, Mosby.)

A

B

DISEASES AND DISORDERS

DIAGNOSIS

Section I

BASIC INFORMATION

WORKUP

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Bursitis, Anserine BASIC INFORMATION DEFINITION Pes anserinus “goose foot” is the insertion of the conjoined tendons into the anteromedial proximal tibia. From anterior to posterior, pes anserinus is made up of the tendons of the sartorius, gracilis, and semitendinosus muscles. Anserine bursitis describes inflammation of the pes anserine bursa underlying the conjoined tendons of the gracilis and semitendinosus muscles and separating them from the head of the tibia.

SYNONYMS Pes anserinus bursitis Anserine tendinitis ICD-9CM CODES 726.61

EPIDEMIOLOGY & DEMOGRAPHICS • Age: common in patients 50 to 80 yr old who suffer from osteoarthritis of the knees • Sex: incidence also higher among obese, elderly women with or without associated knee osteoarthritis, perhaps because of broader female pelvis and greater angulation of the legs at the knees, placing additional stresses on these structures • Association with diabetes even after adjusting to weight • The presence of underlying knee osteoarthritis should not dissuade clinicians from diagnosing anserine bursitis as a potential cause for knee pain

PHYSICAL FINDINGS & CLINICAL PRESENTATION SYMPTOMS • Pain involves the distal inner knee on ascending or descending stairs. • Pain (same location) occurs when arising from a seated position or at night. Patients usually do not experience pain with walking on level surfaces. • Pain may be acute or chronic. • Intensity of the pain is variable but can be severe, unrelenting, and disabling. • Symptoms of anserine bursitis may be associated with certain athletic activities such as running, tennis, or breaststroke swimming. PHYSICAL FINDINGS • Hallmark is tenderness over the proximal medial tibia at the insertion of the conjoined tendons of the pes anserinus, approximately 2 to 3 cm below the anteromedial joint margin of the knee, and no tenderness at the joint line itself unless other conditions are present. • Local swelling may be noted but the bursa is not palpable unless effusion and thickening are present.

• Palpable crepitus is occasionally observed. • Anserine bursitis occurs more frequently on the right side than the left, and one third of patients have bilateral involvement. • If swelling or tenderness can be palpated more proximally along the pes anserinus tendons, tendinitis may be present.

ETIOLOGY • Anserine bursitis is an inflammatory condition of the medial knee resulting from trauma, overuse, or degenerative processes and often coexists with other knee disorders. • The sartorius, gracilis, and semitendinosus muscles are primary flexors of the knee. These three muscles also protect the knee against rotary and valgus strain. Bursitis may result from stress to this area such as when an obese individual with anatomic deformity from arthritis ascends or descends stairs. • Degenerative joint disease (DJD) of the knee is often associated with bursitis. Up to 75% of patients with DJD may have symptoms of anserine bursitis. • Obesity is associated with anserine bursitis, most notably in elderly women. • Flat feet may predispose patients to anserine bursitis.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Fibromyalgia • Hamstring strain • Medial collateral and lateral collateral ligament injury • Osteoarthritis of the knee • Patellofemoral syndrome • Prepatellar bursitis and inflammation of other knee bursae • Medial meniscus injury or degenerative tear • Inflammatory arthritis involving the knee • Sarcomas and other tumors involving the knee structures • Painful neuropathies

WORKUP • Usually the diagnosis is based on history and physical exam. Response to treatment (e.g., bursa injection) is also helpful. • Rarely, bursa aspiration may be indicated if infection or crystal-induced inflammation is suspected. When done, the aspirate should be analyzed for cell count, culture and sensitivity, and crystals. In this scenario, it may be appropriate also to perform a CBC and ESR.

IMAGING STUDIES • Imaging is rarely indicated.

• X-ray may show osteoarthritis. This finding does not make the diagnosis of anserine bursitis any more or less likely.

TREATMENT NONPHARMACOLOGIC THERAPY • Physical therapy  Stretching and strengthening of the adductor and quadriceps muscles and stretching of the hamstrings • Rest but avoid disuse atrophy • Weight loss if overweight • Ice massage for 10 minutes three times daily • Ultrasound therapy • Cushion between thighs or knee at night if nocturnal pain

PHARMACOLOGIC THERAPY • Nonsteroidal antiinflammatory drugs are not very effective. • Topical capsaicin may be applied (use 0.075% strength tid). • Intrabursal injection of an anesthetic (e.g., 1 to 2 cc lidocaine 1% or 2%) combined with a corticosteroid (e.g., 1 to 2 cc of triamcinolone 20 to 40 mg) using a 22- or 23-gauge needle is effective in confirming the diagnosis (pain relief occurs in less than 5 minutes if the diagnosis is correct and the medication is injected in the bursa). The injection site is usually the area of maximum tenderness, but it should be located 1 to 2 inches below the anteromedial proximal end of the tibia to be consistent with the location of the anserine bursa. Some clinicians limit the number of intrabursal steroid injections to three or four per year, but this is based on recommendations that apply to intraarticular injection. A successful injection provides 3 to 4 weeks of symptom relief and may be repeated when symptoms recur.

REFERRAL • Referral to orthopedic surgeon in case of medical treatment failure. Surgery for bursectomy or removal of exostoses is rarely necessary. • Prognosis remissions and recurrences are common and often parallel the progression of the underlying osteoarthritis.

SUGGESTED READINGS Fireman HH: Don’t forget anserine bursitis, CMAJ 165(10):1300, 2001. Gnanadesigan N, Smith RL: Knee pain: osteoarthritis or anserine bursitis? J Am Med Dir Assoc 4(3):164-166, 2003. AUTHOR: TOM J. WACHTEL, M.D.

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Carpal Tunnel Syndrome BASIC INFORMATION

ICD-9CM CODES 354.0 Carpal tunnel syndrome

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENT AGE: Any age (bilateral up to 50%) PREVALENT SEX: Females are affected two to five times as often as males

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Nocturnal pain • Occasional median nerve sensory impairment (often only index and long fingers) • Positive Tinel’s sign at wrist (tapping over the median nerve on the flexor surface of the wrist produces a tingling sensation radiating from the wrist to the hand) • Positive Phalen’s test (reproduction of symptoms after 1 min of gentle, unforced wrist flexion)

ETIOLOGY • Idiopathic in most cases • Space-occupying lesions in carpal tunnel (tenosynovitis, ganglia, aberrant muscles) • Job-related mechanical overuse may be a risk factor • Traumatic injuries to wrist

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

Cervical radiculopathy Chronic tendinitis Vascular occlusion Reflex sympathetic dystrophy Osteoarthritis Other arthritides Other entrapment neuropathies

IMAGING STUDIES Routine roentgenograms may be helpful in establishing cause or ruling out other conditions.

Nerve conduction velocity tests and electromyography are useful in establishing the diagnosis and ruling out other syndromes.

TREATMENT ACUTE GENERAL Rx • • • •

Elimination of repetitive trauma Occupational splints or braces NSAIDs Injection of carpal canal on ulnar side of palmaris longus tendon at wrist flexor crease (avoiding median nerve) • Low-dose oral corticosteroids (e.g., prednisolone 20 mg qd for 2 wk, followed by 10 mg qd for 2 more wk) are also effective for symptom relief in selected patients • Stretching exercises

DISPOSITION Prognosis is variable. Some cases resolve spontaneously. Relief from local injection appears transient and symptoms recur in the majority of cases following injection.

REFERRAL Surgical referral in cases of failed medical management or signs of motor weakness. Results of surgery usually excellent with return to full activity in 4-6 weeks.

SUGGESTED READINGS CARPAL TUNNEL SYNDROME

Median nerve in carpal tunnel Tapping produces paresthesias (Tinel's sign)

FIGURE 1-10 Distribution of pain and/or paresthesias (dark-shaded area) when the median nerve is compressed by swelling in the wrist (carpal tunnel). (From Arnett FC: Rheumatoid arthritis. In Andreoli TE [ed]: Cecil essentials of medicine, ed 4, Philadelphia, 1997, WB Saunders.)

Dias JJ, Burke FD et al: Carpal tunnel syndrome and work, J Hand Surg 29:329, 2004. Geoghegan JM, Clark DI et al: Risk factors in carpal tunnel syndrome, J Hand Surg 29:315, 2004. Gerritsen AM et al: Splinting vs surgery in the treatment of carpal tunnel syndrome, JAMA 288:1245, 2002. Goodyear-Smith F, Arroll B: What can family physicians offer patients with carpal tunnel syndrome other than surgery? A systematic review of nonsurgical management, Ann Fam Med 2:267, 2004. Hui AC, Wong SM et al: Long-term outcome of carpal tunnel syndrome after conservative treatment, Int J Clin Pract 58:337, 2004. Katz JN, Simmons BP: Carpal tunnel syndrome, N Engl J Med 346:1807, 2002. Lee DH, Claussen GC, Oh S: Clinical nerve conduction and needle electromyography studies, J Am Acad Orthop Surg 12:276, 2004. Shum C et al: The role of fleyor tenosynovectomy in the operative treatment of carpal tunnel syndrome, J Bone Joint Surg 84(A):221, 2002. Vjera AJ: Management of carpal tunnel syndrome, Am Fam Physician 68:265, 2003. AUTHOR: LONNIE R. MERCIER, M.D.

DISEASES AND DISORDERS

Carpal tunnel syndrome is an entrapment neuropathy involving the median nerve at the wrist (Fig. 1-10). It is the most common entrapment neuropathy in the upper extremity.

ELECTRODIAGNOSTIC STUDIES Section I

DEFINITION

• Carpal compression test: Pressure with the examiner’s thumb over the patient’s carpal tunnel for 30 sec elicits symptoms • Thenar atrophy in long-standing cases

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Cataracts

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BASIC INFORMATION

DIAGNOSIS

CHRONIC Rx

DEFINITION

DIFFERENTIAL DIAGNOSIS

Cataracts are the clouding and opacification of the normally clear crystalline lens of the eye. The opacity may occur in the cortex, the nucleus of the lens, or the posterior subcapsular region, but it is usually in a combination of areas.

• Corneal lesions • Retinal lesions, detached retina, tumors • Vitreous disease, chronic inflammation

• Change glasses as cataracts develop. • Myopia is common, and glasses can be adjusted until surgery is contemplated.

SYNONYMS Congenital cataracts (e.g., from rubella) Metabolic cataracts (e.g., caused by diabetes) Collagen vascular disease cataracts (caused by lupus) Hereditary cataracts Age-related senile cataracts Traumatic cataracts Toxic or drug-induced cataracts (e.g., caused by steroids) ICD-9CM CODES 366 Cataract

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): Highest cause of treatable blindness; cataract removal is the most frequent surgical procedure in patients 65 yr old (1.3 million operations/yr, with an annual cost of approximately $3 billion). By year 2020 expect over 30 million Americans to have cataracts. Of Americans 40, 20.5 million (17.2%) have cataracts. Of these, 5% have had surgery. PREDOMINANT AGE: Elderly; some stage of cataract development is present in 50% of persons 65 to 74 yr old and 65% of those 75 yr old. Lens clouding begins at 39 to 40 yr old and then usually progresses either slowly or rapidly depending upon individual and health. GENETICS: Hereditary with such syndromes as homocystinuria, diabetes, myotonic dystrophy

WORKUP • Complete eye examination, including slit lamp examination, funduscopic examination, and brightness acuity testing • Complete physical exam for other underlying causes

LABORATORY TESTS • Fasting glucose • Consider Collagen vascular and other metabolic diseases in selected cases

TREATMENT There is no evidence that antioxidants or drugs will slow down or help cataracts.

NONPHARMACOLOGIC THERAPY • Wait until vision is compromised before doing surgery. • Surgery is indicated when corrected visual acuity in the affected eye is 20/30 in the absence of other ocular disease; however, surgery may be justified when visual acuity is better in specific situations (especially disabling glare, monocular diplopia). Surgery indicated when vision in one eye is greatly different from other and affects patient’s life.

DISPOSITION Refer if sight compromised or inflamed red eye.

REFERRAL Refer to ophthalmologist for evaluation extraction when vision is compromised (see Nonpharmacologic Therapy).

PEARLS & CONSIDERATIONS Patients want to know five things about cataracts: 1. chance for vision improvement 2. when will vision improve 3. risk from surgery 4. effect of surgery 5. types of complications

COMMENTS Success rate with surgery is 95% to 98%.

SUGGESTED READINGS Congdon N et al: Prevalence of cataract and pseudophakia/aphakia among adults in the US, Arch Ophthalmol 122(4)487, 2004. Consultation section: Cataract surgical problem, J Cataract Refract Surg 28:577, 2002. Solomon R, Donninfeld ED: Recent advances and future frontiers in treating age-related cataracts, JAMA 290:248, 2003. Wong TY et al: Relation of ocular trauma to cortical, nuclear and posterior subcapsular cataracts, Br J Ophthalmol 86:152, 2002. AUTHOR: MELVYN KOBY, M.D.

ACUTE GENERAL Rx None necessary except when acute glaucoma or inflammation occurs.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Cloudiness and opacification of the crystalline lens of the eye (Fig. 1-11)

1

ETIOLOGY • • • • • • • • •

Heredity Trauma Toxins Age-related Drug-related Congenital Inflammatory Diabetes Collagen vascular disease

FIGURE 1-11 The central location of a posterior subcapsular cataract (1). (From Palay D [ed]: Ophthalmology for the primary care physician, St Louis, 1997, Mosby.)

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Cellulitis BASIC INFORMATION Cellulitis is a superficial inflammatory condition of the skin. It is characterized by erythema, warmth, and tenderness of the area involved. Erysipelas (cellulitis generally secondary to group A -hemolytic streptococci) ICD-9CM CODES 682.9 Cellulitis

EPIDEMIOLOGY & DEMOGRAPHICS • Occurs most frequently in diabetics, immunocompromised hosts, and patients with venous and lymphatic compromise. • Frequently found near skin breaks (trauma, surgical wounds, ulcerations, tinea infections). Edema, animal or human bites, subadjacent osteomyelitis, and bacteremia are potential sources of cellulitis

PHYSICAL FINDINGS & CLINICAL PRESENTATION Variable with the causative organism • Erysipelas: superficial-spreading, warm, erythematous lesion distinguished by its indurated and elevated margin; lymphatic involvement and vesicle formation are common. • Staphylococcal cellulitis: area involved is erythematous, hot, and swollen; differentiated from erysipelas by nonelevated, poorly demarcated margin; local tenderness and regional adenopathy are common; up to 85% of cases occur on the legs and feet. • Vibrio vulnificus: larger hemorrhagic bullae, cellulitis, lymphadenitis, myositis; often found in critically ill patients in septic shock.

ETIOLOGY • Group A -hemolytic streptococci (may follow a streptococcal infection of the upper respiratory tract) • Staphylococcal cellulitis • Vibrio vulnificus: higher incidence in patients with liver disease (75%) and in immunocompromised hosts (corticosteroid use, diabetes mellitus, leukemia, renal failure) • Erysipelothrix rhusiopathiae: common in people handling poultry, fish, or meat • Aeromonas hydrophila: generally occurring in contaminated open wound in fresh water • Fungi (Cryptococcus neoformans): immunocompromised granulopenic patients

DIFFERENTIAL DIAGNOSIS • • • • • • • • • • • • •

Angiodema Necrotizing fasciitis DVT Peripheral vascular insufficiency Paget’s disease of the breast Thrombophlebitis Acute gout Psoriasis Candida intertrigo Pseudogout Osteomyelitis Insect bite Fixed drug eruption

WORKUP Physical examination and laboratory evaluation

LABORATORY TESTS • Gram stain and culture (aerobic and anaerobic) 1. Aspirated material from: a. Advancing edge of cellulitis b. Any vesicles 2. Swab of any drainage material 3. Punch biopsy (in selected patients) • Blood cultures in hospitalized patients, in patients who have cellulitis superimposed on lymphedema, in patients with buccal or periorbital cellulitis, and in patients suspected of having a salt-water or fresh-water source of infection. Bacteremia is uncommon in cellulitis (positive blood cultures in only 4% of patients) Despite the previous measures, the cause of cellulitis remains unidentified in most patients.

IMAGING STUDIES Radiologic examination is unnecessary in most cases of cellulitis. CT or MRI in patients with suspected necrotizing fasciitis (deep-seated infection of the subcutaneous tissue that results in the progressive destruction of fascia and fat).

TREATMENT NONPHARMACOLOGIC THERAPY Immobilization and elevation of the involved limb. Cool sterile saline dressings to remove purulence from any open lesion. Support stockings in patients with peripheral edema.

Erysipelas • PO: dicloxacillin 500 mg PO q6h • IV: cefazolin 1 g q6-8h or nafcillin 1.0 or 1.5 g IV q4-6h NOTE: Use erythromycin, cephalosporins, clindamycin, or vancomycin in patients allergic to penicillin. Staphylococcus cellulitis • PO: dicloxacillin 250 to 500 mg qid • IV: nafcillin, 1 to 2 g q4-6h • Cephalosporins (cephalothin, cephalexin, cephradine) also provide adequate antistaphylococcal coverage except for MRSA • Use vancomycin 1.0-2.0 g IV qd or linezolid 0.6 g IV q12h in patients allergic to penicillin or cephalosporins and in patients with methicillin-resistant S. aureus (MRSA). Daptomycin (Cubicin), a cyclic lipopeptide can be used as an alternative to vancomycin for complicated skin and skin structure infections. Usual dose is 4 mg/kg IV given over 30 min every 24 hr. Linezolide (Zyvox) can also be used (400 mg PO bid for 10 days). Vibrio vulnificus • Doxycycline 100 mg IV or PO bid / third-generation cephalosporin. Ciprofloxacin is an alternative antibiotic • IV support and admission into ICU (mortality rate 50% in septic shock) Erysipelothrix • Penicillin Aeromonas hydrophila • Aminoglycosides • Chloramphenicol • Complicated skin and skin structure infections in hospitalized patients can be treated with daptomycin (Cubicin) 4 mg/kg IV every 24 hr

DISPOSITION Prognosis is good with prompt treatment.

REFERRAL For surgical debridement in addition to antibiotics in patients with suspected necrotizing fasciitis

PEARLS & CONSIDERATIONS Bilateral distal leg erythema is more likely venous insufficiency and stasis dermatitis than cellulitis.

SUGGESTED READING Swartz MN: Cellulitis, N Engl J Med 350:904, 2004. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

SYNONYMS

DIAGNOSIS

ACUTE GENERAL Rx Section I

DEFINITION

• Gram-negative rods (Serratia, Enterobacter, Proteus, Pseudomonas): immunocompromised or granulopenic patients

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Cholelithiasis BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Cholelithiasis is the presence of stones in the gallbladder.

• • • • • • •

SYNONYMS Gallstones ICD-9CM CODES 574.2 Calculus of the gallbladder without mention of cholecystitis 574.0 Calculus of the gallbladder with acute cholecystitis

EPIDEMIOLOGY & DEMOGRAPHICS • Gallstone disease can be found in 20 million Americans. Of these, 2% to 3% (500,000 to 600,000) are treated with cholecystectomies each year. • Annual medical expenditures for gallbladder surgeries in the U.S. exceed $5 billion. • Incidence of gallbladder disease increases with age. Highest incidence is in the fifth and sixth decades. Predisposing factors for gallstones are female sex, family history of gallstones, obesity, ileal disease, diabetes mellitus, rapid weight loss, estrogen replacement therapy. • Patients with gallstones have a 20% chance of developing biliary colic or its complications at the end of a 20-yr period.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Physical examination is entirely normal unless patient is having a biliary colic; 80% of gallstones are asymptomatic. • Typical symptoms of obstruction of the cystic duct include intermittent, severe, cramping pain affecting the RUQ. • Pain occurs mostly at night and may radiate to the back or right shoulder. It can last from a few minutes to several hours.

ETIOLOGY • 75% of gallstones contain cholesterol and are usually associated with obesity, female sex, diabetes mellitus; mixed stones are most common (80%), pure cholesterol stones account for only 10% of stones. • 25% of gallstones are pigment stones (bilirubin, calcium, and variable organic material) associated with hemolysis and cirrhosis. These tend to be black pigment stones that are refractory to medical therapy. • 50% of mixed-type stones are radiopaque.

PUD GERD IBD Pancreatitis Neoplasms Nonulcer dyspepsia Functional bowel disease

LABORATORY TESTS Generally normal unless patient has biliary obstruction (elevated alkaline phosphatase, bilirubin).

IMAGING STUDIES • Ultrasound of the gallbladder will detect small stones and biliary sludge (sensitivity, 95%; specificity, 90%); the presence of dilated gallbladder with thickened wall is suggestive of acute cholecystitis. • Nuclear imaging (HIDA scan) can confirm acute cholecystitis (90% accuracy) if gallbladder does not visualize within 4 hr of injection and the radioisotope is excreted in the common bile duct.

TREATMENT NONPHARMACOLOGIC THERAPY Lifestyle changes (avoidance of diets high in polyunsaturated fats, weight loss in obese patients—however, avoid rapid weight loss)

ACUTE GENERAL Rx • The management of gallstones is affected by the clinical presentation. • Asymptomatic patients do not require therapeutic intervention. • Surgical intervention is generally the ideal approach for symptomatic patients. Laparoscopic cholecystectomy is generally preferred over open cholecystectomy because of the shorter recovery period. • Laparoscopic cholecystectomy after endoscopic sphincterectomy is recommended for patients with common bile duct stones and residual gallbladder stones. Where possible, single-stage laparoscopic treatments with removal of duct stones and cholecystectomy during the same procedure are preferable. • Patients who are not appropriate candidates for surgery because of coexisting illness or patients who refuse surgery can be treated with oral bile salts: ursodiol (Actigall) 8 to 10 mg/kg/day

in two to three divided doses for 16 to 20 mo, or chenodiol (Chenix) 250 mg bid initially, increasing gradually to a dose of 60 mg/kg/day. Candidates for oral bile salts are patients with cholesterol stones (radiolucent, noncalcified stones), with a diameter of 15 mm and having three or fewer stones. Candidates for medical therapy must have a functioning gallbladder and must have absence of calcifications on CT scans. • Direct solvent dissolution with methyl tert-butyl ether (MTBE) can be used in patients with multiple stones with diameter 3 cm; this method should be used only by physicians experienced with contact dissolution. Administration of the solvent is either through percutaneous transhepatic placement of a catheter into the gallbladder or endoscopic retrograde catheter placement with subsequent continuous infusion and aspiration of the solvent either manually or by automatic pump system. MTBE is a powerful cholesterol solvent and can dissolve stones in a few hours (90% dissolution over a 2-hr infusion). • Extracorporeal shock wave lithotripsy (ESWL) is another form of medical therapy. It can be used in patients with stone diameter of 3 cm and having three or fewer stones.

DISPOSITION • Recurrence rate after bile acid treatment is approximately 50% in 5 yr. Periodic ultrasound is necessary to assess the effectiveness of treatment. • Gallstones recur after dissolution therapy with MTBE in 40% of patients within 5 yr. • Following extracorporeal shock wave lithotripsy, stones recur in approximately 20% of patients after 4 yr. • Patients with at least one gallstone 5 mm in diameter have a greater than fourfold increased risk of presenting with acute biliary pancreatitis. A policy of watchful waiting in such cases is generally unwarranted. • A potential serious complication of gallstones is acute cholangitis. ERCP and endoscopic sphincterectomy (EC) followed by interval laparoscopic cholecystectomy is effective in acute cholangitis. • Patients with functional bowel disease and co-existing gall stones may continue to have symptoms after cholecystectomy. AUTHOR: FRED F. FERRI, M.D.

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Chronic Obstructive Pulmonary Disease BASIC INFORMATION

SYNONYMS COPD Emphysema Chronic bronchitis ICD-9CM CODES 496 COPD 492.8 Emphysema

EPIDEMIOLOGY & DEMOGRAPHICS • COPD affects 16 million Americans and is responsible for 80,000 deaths/yr. • COPD is the fourth leading cause of death in the U.S. and is expected to become the third leading cause of death by 2020. • Highest incidence is in males 40 yr. • 16 million office visits, 500,000 hospitalizations, and $18 billion in direct health care costs annually can be attributed to COPD.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Blue bloaters (chronic bronchitis): peripheral cyanosis, productive cough, tachypnea, tachycardia • Pink puffers (emphysema): dyspnea, pursed-lip breathing with use of accessory muscles for respiration, decreased breath sounds • Possible wheezing in both patients with chronic bronchitis and emphysema

ETIOLOGY • Tobacco exposure • Occupational exposure to pulmonary toxins (e.g., cadmium) • Atmospheric pollution • -1 antitrypsin deficiency (rare; 1% of COPD patients)

IMAGING STUDIES Chest x-ray examination: • Hyperinflation with flattened diaphragm, tending of the diaphragm at the rib, and increased retrosternal chest space • Decreased vascular markings and bullae in patients with emphysema • Thickened bronchial markings and enlarged right side of the heart in patients with chronic bronchitis ECG and echocardiogram may be appropriate to diagnose associated heart disease (also caused by smoking).

TREATMENT

DIAGNOSIS

NONPHARMACOLOGIC THERAPY

DIFFERENTIAL DIAGNOSIS

• Weight loss in patients who are obese • Avoidance of tobacco use and elimination of air pollutants • Supplemental oxygen, usually through a nasal cannula, to ensure oxygen saturation 90% measured by pulse oximetry

• • • • • • • • •

CHF Asthma Respiratory infections Bronchiectasis Pulmonary embolism Sleep apnea, obstructive Pulmonary fibrosis Other restrictive lung diseases Anxiety disorders

WORKUP Chest x-ray examination, pulmonary function testing, blood gases

LABORATORY TESTS • CBC may reveal leukocytosis with “shift to the left” during acute exacerbation. • Sputum may be purulent with bacterial respiratory tract infections. Sputum staining and cultures are usually reserved for cases that are refractory to antibiotic therapy. • ABGs: hypercapnia and hypoxemia may be present. • Pulmonary function testing (PFT): the primary physiologic abnormality in COPD is an accelerated decline in forced expiratory volume in one second (FEV1) from the normal rate in adults over 30 yr of age of approximately 30 ml/yr to nearly 60 ml/yr. PFT results in COPD reveal abnormal diffusing capacity, increased total lung capacity or residual volume, and fixed reduction in FEV1 in patients with emphysema and normal diffusing capacity and reduced FEV1 in patients with chronic bronchitis. Patients with COPD can generally be distinguished from asthmatics by their incomplete response to albuterol (change in FEV1 200 ml and 12%) and absence of an

ACUTE EXACERBATION • Acute exacerbation of COPD can be treated with: 1. Pulmonary toilet: careful nasotracheal suction is indicated in patients with excessive secretions and inability to expectorate. Mechanical percussion of the chest as applied by a physical or respiratory therapist is ineffective with acute exacerbations of COPD. 2. Aerosolized -agonists (e.g., albuterol nebulizer solution 5% 0.3 ml or albuterol nebulized 5% solution 2.5 to 5 mg). 3. Anticholinergic agents, which have equivalent efficacy to inhaled adrenergic agonists. Inhalant solution of ipratropium bromide 0.5 mg can be administered every 4 to 8 hours. Tiotropium bromide (Spiriva) has a much longer duration of action than ipratropium and produces a more sustained response over time than long-acting 2-agonists. Usual dose is inhalation of one capsule daily with a breath-activated dry powder inhalation device (HandiHaler). 4. Short courses of systemic corticosteroids, which have been shown to improve spirometric and clinical outcomes. In the hospital setting give IV methylprednisolone 50- to 100-mg bolus, then q6-8h; taper as soon as possible. In the outpatient

DISEASES AND DISORDERS

Chronic obstructive pulmonary disease (COPD) is a disorder characterized by the presence of airflow limitation that is not fully reversible. COPD includes emphysema, characterized by loss of lung elasticity and destruction of lung parenchyma with enlargement of air spaces, and chronic bronchitis, characterized by obstruction of small airways and productive cough greater than 3 months’ duration for more than 2 successive years. Patients with COPD are classically subdivided in two major groups based on their appearance: 1. Blue bloaters are patients with chronic bronchitis; the name is derived from the bluish tinge of the skin (secondary to chronic hypoxemia); chronic cough with production of large amounts of sputum is characteristic. 2. Pink puffers are patients with emphysema; they are often cachectic and have pink skin color (adequate oxygen saturation); shortness of breath may be manifested by pursed-lip breathing and use of accessory muscles of respiration. Both may cause hypercapnia and both often coexist.

abnormal bronchoconstrictor response to methacholine or other stimuli. However, nearly 40% of patients with COPD respond to bronchodilators.

Section I

DEFINITION

• Features of both chronic bronchitis and emphysema in many patients with COPD • Acute exacerbation of COPD is mainly a clinical diagnosis and generally manifests with worsening dyspnea, increase in sputum purulence, and increase in sputum volume • Complications often involve rightsided heart failure (cor pulmonale), sometimes precipitated by arrhythmias such as atrial fibrillation or multifocal atrial tachycardia

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Chronic Obstructive Pulmonary Disease

•

•

•

•

setting, oral prednisone 40 mg/day initially, decreasing the dose by 10 mg every other day is generally effective. 5. Judicious oxygen administration (hypercapnia and further respiratory compromise may occur after high-flow oxygen therapy). Use of a Venturi-type mask delivering an inspired oxygen fraction of 24% to 28% is preferred to nasal cannula. 6. Noninvasive positive pressure ventilation, which when delivered by a facial or nasal mask may obviate the need for intratracheal intubation. 7. Inhaled corticosteroids, whose role in COPD is controversial. Although some trials have demonstrated mild improvement in patients’ symptoms and decreased frequency of exacerbations, most pulmonologists believe that these drugs are ineffective in most patients with COPD but should be considered for patients with moderate to severe airflow limitation who have persistent symptoms despite optimal bronchodilator therapy. 8. IV aminophylline administration, which is controversial. When used, serum levels should be closely monitored to minimize risks of tachyarrhythmias. Cor pulmonale or comorbid heart failure may be exacerbated by hypoxia and must be treated along with the pulmonary management (see Heart Failure, Congestive in Section I). Antibiotics are indicated in suspected respiratory infection (e.g., increased purulence and volume of phlegm). 1. Haemophilus influenzae and Streptococcus pneumoniae are frequent causes of acute bronchitis. 2. Oral antibiotics of choice are azithromycin, levofloxacin, amoxicillin-clavulanate, and cefuroxime. 3. The use of antibiotics is beneficial in exacerbations of COPD presenting with increased dyspnea and sputum purulence (especially if the patient is febrile). Guaifenesin may improve cough symptoms and mucus clearance; however, mucolytic medications are generally ineffective. Their benefits may be greatest in patients with more advanced disease. Intubation and mechanical ventilation may be necessary if above measures fail to provide improvement.

CHRONIC Rx • Inhaled -agonists, short-acting agents such as albuterol can be used on a schedule (e.g., two to four puffs q46h). • Inhaled -agonists, long-acting agents such as salmeterol (Serevent) can be given twice daily. • Anticholinergic short-acting agent such as ipratropium (Atrovent) can be used on a schedule or prn, alone, or in combination with the -agonist albuterol (Combivent). • Anticholinergic long-acting agent tiotropium (Spiriva): one inhalation (18 g dry powder) qd may be more effective than ipratropium. • An inhaled glucocorticoid drug such as fluticasone is given twice daily alone or in combination with -agonists or anticholinergic drugs (e.g., Advair combines fluticasone and salmeterol). • Elderly patients may be unable to coordinate their breathing and hand motion or may otherwise be unable to operate a handheld metered dose inhaler. If such treatment is prescribed, the physician must educate and observe the patient using this therapy. A spacer may be helpful and should be prescribed routinely. Elderly patients often do better with a nebulizer than with a handheld aerosol delivery system. • Pulmonary rehabilitation may improve exercise capacity, reduce dyspnea, reduce hospitalizations, and enhance quality of life. • Lung-volume-reduction surgery in patients with severe upper-lobe emphysema can improve exercise capacity, pulmonary function, and quality of life but not overall survival.

DISPOSITION • Following the initial episode of respiratory failure, 5-yr survival is approximately 25%. • Development of cor pulmonale or hypercapnia and persistent tachycardia are poor prognostic indicators.

PEARLS & CONSIDERATIONS COMMENTS • All patients with COPD should receive pneumococcal vaccine and yearly influenza vaccine.

• In assessing the severity of COPD, the FEV1 is limited by the fact that it does not take into account the systemic manifestations of COPD. The BODE index (body-mass index [B], degree of obstruction [O], dyspnea [D], and exercise capacity [E]) has been proposed as a multidimensional scale to better assess the morbidity and mortality associated with COPD. It is better than the FEV1 at predicting the risk of death from any cause and from respiratory causes among patients with COPD. • Osteoporosis is often associated with COPD; consider DXA.

SUGGESTED READINGS Aaron SD et al: Outpatient oral prednisone after emergency treatment of chronic obstructive pulmonary disease, N Engl J Med 348:2618, 2003. Anthonisen NR et al: Smoking and lung function of Lung Health Study participants after 11 years, Am J Resp Crit Care Med 166:675679, 2002. Celli B et al: The body-mass index, airflow obstruction, dyspnea, and exercise capacity index in chronic obstructive pulmonary disease, N Engl J Med 350:1005-1012, 2004. Hogg JC et al: The nature of small-airway obstruction in chronic obstructive pulmonary disease, N Engl J Med 350:2645-2653, 2004. Man PS et al: Contemporary management of chronic obstructive pulmonary disease, clinical applications, JAMA 290:2313, 2003. National Emphysema Treatment Trial Research Group: a randomized trial comparing lung-volume-reduction surgery with medical therapy for severe emphysema, N Engl J Med 348:2059-2073, 2003. Sethi S et al: New strains of bacteria and exacerbations of chronic obstructive pulmonary disease, N Engl J Med 347:465, 2002. Sin DD et al: Contemporary management of chronic obstructive pulmonary disease, a scientific review, JAMA 290:2301, 2003. Stoller JK: Acute exacerbations of chronic obstructive pulmonary disease, N Engl J Med 346:988, 2002. Sutherland ER, Cherniak RM: Management of chronic obstructive pulmonary disease, N Engl J Med 350:2689-2697, 2004. Vincken W et al: Improved health outcomes in patients with COPD during 1 year’s treatment with tiotropium, Eur Respir J 19:209216, 2002. AUTHORS: FRED F. FERRI, M.D., and TOM J. WACHTEL, M.D.

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Cirrhosis BASIC INFORMATION

ICD-9CM CODES 571.5 Cirrhosis of the liver 571.2 Cirrhosis of the liver secondary to alcohol

EPIDEMIOLOGY & DEMOGRAPHICS • Cirrhosis is the eleventh leading cause of death in the U.S. (death rate 9 deaths/100,000 persons/yr). • Alcohol abuse and viral hepatitis are the major causes of cirrhosis in the U.S. • Nonalcoholic fatty liver disease (NAFLD) is becoming recognized as an important cause of cirrhosis, and its prevalence is increasing.

ETIOLOGY

• Decreased Hgb and Hct, elevated MCV, increased BUN and creatinine (the BUN may also be “normal” or low if the patient has severely diminished liver function), decreased sodium (dilutional hyponatremia), decreased potassium (as a result of secondary aldosteronism or urinary losses). Evaluation of renal function should also include measurement of urinary sodium and urinary protein from a 24-hr urine collection. • Decreased glucose in a patient with liver disease indicating severe liver damage • Other laboratory abnormalities: 1. Alcoholic hepatitis and cirrhosis: there may be mild elevation of ALT and AST, usually 500 IU; AST  ALT (ratio 2:3). 2. Extrahepatic obstruction: there may be moderate elevations of ALT and AST to levels 500 IU. 3. Viral, toxic, or ischemic hepatitis: there are extreme elevations (500 IU) of ALT and AST. 4. Transaminases may be normal despite significant liver disease in patients with jejunoileal bypass operations or hemochromatosis or after methotrexate administration. 5. Alkaline phosphatase elevation can occur with extrahepatic obstruction, primary biliary cirrhosis, and primary sclerosing cholangitis. 6. Serum LDH is significantly elevated in metastatic disease of the liver; lesser elevations are seen with hepatitis, cirrhosis, extrahepatic obstruction, and congestive hepatomegaly. 7. Serum -glutamyl transpeptidase (GGTP) is elevated in alcoholic liver disease and may also be elevated with cholestatic disease (primary biliary cirrhosis, primary sclerosing cholangitis). 8. Serum bilirubin may be elevated; urinary bilirubin can be present in hepatitis, hepatocellular jaundice, and biliary obstruction. 9. Serum albumin: significant liver disease results in hypoalbuminemia. 10. Prothrombin time: an elevated PT in patients with liver disease indicates severe liver damage and poor prognosis. 11. Presence of hepatitis B surface antigen implies acute or chronic hepatitis B. 12. Presence of antimitochondrial antibody suggests primary biliary cirrhosis, chronic hepatitis.

• Alcohol abuse • Nonalcoholic steatohepatitis (NASH) • Secondary biliary cirrhosis, obstruction of the common bile duct (stone, stricture, pancreatitis, neoplasm, sclerosing cholangitis) • Drugs (e.g., acetaminophen, isoniazid, methotrexate, methyldopa) • Hepatic congestion (e.g., CHF, constrictive pericarditis, tricuspid insufficiency, thrombosis of the hepatic vein, obstruction of the vena cava) • Primary biliary cirrhosis • Hemochromatosis • Chronic hepatitis B or C • Wilson’s disease • -1 antitrypsin deficiency • Infiltrative diseases (amyloidosis, glycogen storage diseases, hemochromatosis) • Nutritional: jejunoileal bypass • Others: parasitic infections (schistosomiasis), idiopathic portal hypertension, congenital hepatic fibrosis, systemic mastocytosis, autoimmune hepatitis, hepatic steatosis, IBD

PHYSICAL FINDINGS & CLINICAL PRESENTATION

DIAGNOSIS

SKIN: Jaundice, palmar erythema (alcohol abuse), spider angiomata, ecchymosis (thrombocytopenia or coagulation factor deficiency), dilated superficial periumbilical vein (caput medusae), increased pigmentation (hemochromatosis), xanthomas (primary biliary cirrhosis), needle tracks (viral hepatitis) EYES: Kayser-Fleischer rings (corneal copper deposition seen in Wilson’s disease; best diagnosed with slit lamp examination), scleral icterus BREATH: Fetor hepaticus (musty odor of breath and urine found in cirrhosis with hepatic failure) CHEST: Possible gynecomastia in men ABDOMEN: Tender hepatomegaly (congestive hepatomegaly), small, nodular liver (cirrhosis), palpable, nontender gallbladder (neoplastic extrahepatic biliary obstruction), palpable spleen (portal hypertension), venous hum auscultated over periumbilical veins (portal hypertension), ascites (portal hypertension, hypoalbuminemia) RECTAL EXAMINATION: Hemorrhoids (portal hypertension), guaiac-positive stools (alcoholic gastritis, bleeding esophageal varices, PUD, bleeding hemorrhoids) GENITALIA: Testicular atrophy in males (chronic liver disease, hemochromatosis) EXTREMITIES: Pedal edema (hypoalbuminemia, failure of right side of the heart), arthropathy (hemochromatosis)

WORKUP In addition to an assessment of liver function, the evaluation of patients with cirrhosis should also include an assessment of renal and circulatory function. Diagnostic workup is aimed primarily at identifying the most likely cause of cirrhosis. The history is extremely important: • Alcohol abuse: alcoholic liver disease • History of hepatitis B (chronic active hepatitis, primary hepatic neoplasm, or hepatitis C) • History of IBD (primary sclerosing cholangitis) • History of pruritus, hyperlipoproteinemia, and xanthomas in an elderly female (primary biliary cirrhosis) • Impotence, diabetes mellitus, hyperpigmentation, arthritis (hemochromatosis) • Neurologic disturbances (Wilson’s disease, hepatolenticular degeneration) • Family history of “liver disease” (hemochromatosis [positive family history in 25% of patients], -1 antitrypsin deficiency) • History of recurrent episodes of RUQ pain (biliary tract disease) • History of blood transfusions, IV drug abuse (hepatitis C) • History of hepatotoxic drug exposure • Coexistence of other diseases with immune or autoimmune features (ITP, myasthenia gravis, thyroiditis, autoimmune hepatitis)

LABORATORY TESTS

DISEASES AND DISORDERS

Cirrhosis is defined histologically as the presence of fibrosis and regenerative nodules in the liver. It can be classified as micronodular, macronodular, and mixed; however, each form may be seen in the same patient at different stages of the disease. Cirrhosis manifests clinically with portal hypertension, hepatic encephalopathy, and variceal bleeding.

• Existence of the metabolic syndrome (obesity, hyperlipidemia, diabetes)

Section I

DEFINITION

NEUROLOGIC: Flapping tremor, asterixis (hepatic encephalopathy), choreoathetosis, dysarthria (Wilson’s disease)

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Cirrhosis 13. Elevated serum copper, decreased serum ceruloplasmin, and elevated 24-hr urine may be diagnostic of Wilson’s disease. 14. Protein immunoelectrophoresis may reveal decreased -1 globulins (-1 antitrypsin deficiency), increased IgA (alcoholic cirrhosis), increased IgM (primary biliary cirrhosis), increased IgG (chronic hepatitis, cryptogenic cirrhosis). 15. An elevated serum ferritin and increased transferrin saturation are suggestive of hemochromatosis. 16. An elevated blood ammonia suggests hepatocellular dysfunction; serial values, however, are generally not useful in following patients with hepatic encephalopathy because there is poor correlation between blood ammonia level and degree of hepatic encephalopathy. 17. Serum cholesterol is elevated in cholestatic disorders. 18. Antinuclear antibodies (ANA) may be found in autoimmune hepatitis. 19. Alpha fetoprotein: levels 1000 pg/ml are highly suggestive of primary liver cell carcinoma. 20. Hepatitis C viral testing identifies patients with chronic hepatitis C infection. 21. Elevated level of serum globulin (especially -globulins), positive ANA test may occur with autoimmune hepatitis.

IMAGING STUDIES • Ultrasonography is the procedure of choice for detection of gallstones, fatty liver, and dilation of common bile ducts. • CT scan is useful for detecting mass lesions in liver and pancreas, assessing hepatic fat content, identifying idiopathic hemochromatosis, early diagnosing of Budd-Chiari syndrome, dilation of intrahepatic bile ducts, and detection of varices and splenomegaly. • Technetium-99m sulfur colloid scanning is occasionally useful for diagnosing cirrhosis (there is a shift of colloid uptake to the spleen, bone

marrow), identifying hepatic adenomas (cold defect is noted), diagnosing Budd-Chiari syndrome (there is increased uptake by the caudate lobe). • ERCP is the procedure of choice for diagnosing periampullary carcinoma, common duct stones; it is also useful in diagnosing primary sclerosing cholangitis. • Percutaneous transhepatic cholangiography (PTC) is useful when evaluating patients with cholestatic jaundice and dilated intrahepatic ducts by ultrasonography; presence of intrahepatic strictures and focal dilation is suggestive of PSC. • Percutaneous liver biopsy is useful in evaluating hepatic filling defects, diagnosing hepatocellular disease or hepatomegaly, evaluating persistently abnormal liver function tests, and diagnosing hemachromatosis, primary biliary cirrhosis, Wilson’s disease, glycogen storage diseases, chronic hepatitis, autoimmune hepatitis, infiltrative diseases, alcoholic liver disease, NASH, drug-induced liver disease, primary or secondary carcinoma, and gauging the severity of fibrosis.

TREATMENT

• Glucocorticoids (prednisone 20 to 30 mg/day initially or combination therapy or prednisone and azathioprine) are useful in autoimmune hepatitis. • Treatment of complications of portal hypertension (ascites, esophagogastric varices, hepatic encephalopathy, and hepatorenal syndrome).

DISPOSITION • Prognosis varies with the etiology of the patient’s cirrhosis and whether there is ongoing hepatic injury. Mortality rate exceeds 80% in patients with hepatorenal syndrome. • Two markers of portal hypertension, thrombocytopenia and splenomegaly, moderately increase the likelihood of large esophageal varices. • If advanced cirrhosis is present and transplantation is not feasible, survival is 1 to 2 yr.

REFERRAL • Hospital admission for bleeding varices, hepatic encephalopathy, or onset of hepatorenal syndrome • Liver transplantation in suitable candidates is the only effective long-term treatment of complications resulting from cirrhosis

NONPHARMACOLOGIC THERAPY

PEARLS & CONSIDERATIONS

Avoid any hepatotoxins (e.g., ethanol, acetaminophen); improve nutritional status.

COMMENTS

GENERAL Rx • Correct any mechanical obstruction to bile flow (e.g., calculi, strictures). • Provide therapy for underlying cardiovascular disorders in patients with cardiac cirrhosis. • Remove excess body iron with phlebotomy and deferoxamine in patients with hemochromatosis. • Remove copper deposits with D-penicillamine in patients with Wilson’s disease. • Long-term ursodiol therapy will slow the progression of primary biliary cirrhosis. It is, however, ineffective in primary sclerosing cholangitis.

Thrombocytopenia and advanced ChildPugh cases are associated with the presence of varices. These factors are useful to identify cirrhotic patients who benefit most from referral for endoscopic screening for varices.

SUGGESTED READINGS Gines P et al: Management of cirrhosis and ascites, N Engl J Med 350:1645, 2004. Ong JP et al: Correlation between ammonia levels and the severity of hepatic encephalopathy, Am J Med 114:189, 2003. AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

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Colorectal Cancer

DEFINITION

• Physical examination may be completely unremarkable. • Digital rectal examination can detect approximately 50% of rectal cancers. • Palpable abdominal masses may indicate metastasis or complications of colorectal carcinoma (abscess, intussusception, volvulus). • Abdominal distention and tenderness are suggestive of colonic obstruction. • Hepatomegaly may be indicative of hepatic metastasis.

Colorectal cancer is a neoplasm arising from the luminal surface of the large bowel: descending colon (40% to 42%), rectosigmoid and rectum (30% to 33%), cecum and ascending colon (25% to 30%), transverse colon (10% to 13%). ICD-9CM CODES 154.0 Colorectal cancer

EPIDEMIOLOGY & DEMOGRAPHICS • Colorectal cancer is the second leading cause of cancer deaths in the U.S. (135,000 new cases and 50,000 deaths/yr). • Peak incidence is in the seventh decade of life. • 50% of rectal cancers are within reach of the examiner’s finger, 50% of colon cancers are within reach of the flexible sigmoidoscope. • Colorectal cancer accounts for 14% of all cases of cancer (excluding skin malignancies) and 14% of all yearly cancer deaths. • Risk factors: 1. Hereditary polyposis syndromes a. Familial polyposis (high risk) b. Gardner’s syndrome (high risk) c. Turcot’s syndrome (high risk) d. Peutz-Jeghers syndrome (low to moderate risk) 2. IBD, both ulcerative colitis and Crohn’s disease 3. Family history of “cancer family syndrome” 4. Heredofamilial breast cancer and colon carcinoma 5. History of previous colorectal carcinoma 6. Women undergoing irradiation for gynecologic cancer 7. First-degree relatives with colorectal carcinoma 8. Age 40 yr 9. Possible dietary factors (diet high in fat or meat, beer drinking, reduced vegetable consumption) 10. Hereditary nonpolyposis colon cancer (HNPCC): autosomal-dominant disorder characterized by early age of onset (mean age of 44 yr) and right-sided or proximal colon cancers, synchronous and metachronous colon cancers, mucinous and poorly differentiated colon cancers; it accounts for 1% to 5% of all cases of colorectal cancer 11. Previous endometrial or ovarian cancer, particularly when diagnosed at an early age

ETIOLOGY Colorectal cancer can arise through two mutational pathways: microsatellite instability or chromosomal instability. Germline genetic mutations are the basis of inherited colon cancer syndromes; an accumulation of somatic mutations in a cell is the basis of sporadic colon cancer.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

Diverticular disease Strictures IBD Infectious or inflammatory lesions Adhesions Arteriovenous malformations Metastatic carcinoma (prostate, sarcoma) • Extrinsic masses (cysts, abscesses)

WORKUP • The clinical presentation of colorectal malignancies is initially vague and nonspecific (weight loss, anorexia, malaise). It is useful to divide colon cancer symptoms into those usually associated with right side of colon and those commonly associated with left side of colon, because the clinical presentation varies with the location of the carcinoma. 1. Right side of colon a. Anemia (iron deficiency secondary to chronic blood loss) b. Dull, vague, and uncharacteristic abdominal pain may be present or patient may be completely asymptomatic c. Rectal bleeding is often missed because blood is mixed with feces d. Obstruction and constipation are unusual because of large lumen and more liquid stools 2. Left side of colon a. Change in bowel habits (constipation, diarrhea, tenesmus, pencilthin stools) b. Rectal bleeding (bright red blood coating the surface of the stool) c. Intestinal obstruction is frequent because of small lumen

• Early diagnosis of patients with surgically curable disease (Dukes’ A, B) is necessary, because survival time is directly related to the stage of the carcinoma at the time of diagnosis. Appropriate screening recommendations are discussed in Section V.

CLASSIFICATION Dukes’ and UICC classification for colorectal cancer: A Confined to the mucosa-submucosa (I) B Invasion of muscularis propria (II) C Local node involvement (III) D Distant metastasis (IV)

LABORATORY TESTS • Positive fecal occult blood test • Newer modalities for early detection of colorectal neoplasms include the detection of mutations in the adenomatous polyposis coli (APC) gene from stool samples • Microcytic anemia • Elevated plasma carcinoembryonic antigen (CEA). CEA should not be used as a screening test for colorectal cancer because it can be elevated in patients with many other conditions (smoking, IBD, alcoholic liver disease). A normal CEA does not exclude the diagnosis of colorectal cancer • Liver function tests

IMAGING STUDIES • Colonoscopy with biopsy (primary assessment tool) • CT scan of abdomen to assist in preoperative staging • Chest x-ray examination to look for evidence of metastatic disease • Air-contrast barium enema only in patients refusing colonoscopy or unable to tolerate colonoscopy

TREATMENT GENERAL Rx • Surgical resection: 70% of colorectal cancers are resectable for cure at presentation; 45% of patients are cured by primary resection. • Radiation therapy is a useful adjunct to fluorouracil and levamisole therapy for stage II or III rectal cancers. • Adjuvant chemotherapy with combination of 5-fluorouracil (5-FU) and levamisole substantially increases cure rates for patients with stage III colon cancer and should be considered standard treatment for all such patients and selected patients with high-risk stage II colon cancer. • Leucovorin (folinic acid) enhances the effect of fluorouracil and is given together with it (FL). When given as adjuvant therapy after a complete resection in stage III disease, FL increases overall

DISEASES AND DISORDERS

PHYSICAL FINDINGS & CLINICAL PRESENTATION

Section I

BASIC INFORMATION

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Colorectal Cancer

•

•

•

•

5-year survival from 51% to 64%. The use of adjuvant FL in stage II disease (no involvement of regional nodes) is controversial because 5-yr overall survival is 80% for treated or untreated patients and the addition of FL only increases the probability of 5-yr diseasefree interval from 72% to 76%. For patients with standard-risk stage III tumors (e.g., involvement of one to three regional lymph nodes), FL alone or FL with oxaliplatin (Eloxatin, an inhibitor of DNA synthesis) are both reasonable choices. Generally reversible peripheral neuropathy is the main side effect of FL plus oxaliplatin. Irinotecan (Camptosar), a potent inhibitor of topoisomerase I, a nuclear enzyme involved in the unwinding of DNA during replication, can be used to treat metastatic colorectal cancer refractory to other drugs, including 5-FU; it may offer a few months of palliation but is expensive and associated with significant toxicity. Oxaliplatin can be used in combination with fluorouracil and leucovorin (FL) for patients with metastatic colorectal cancer whose disease has recurred or progressed despite treatment with fluorouracil/leucovorin plus irinotecan. FL plus oxaliplatin should be considered for high-risk patients with stage III cancers (e.g., 3 involved regional nodes [N2] or tumor invasion beyond the serosa [T4 lesion]). The monoclonal antibodies cetuximab (Erbitux) and bevacizumab (Avastatin) have also been approved by the FDA for advanced colorectal cancer. Bevacizumab is an angiogenesis inhibitor that binds and inhibits the activity of human vascular endothelial growth factor (VEGF). Cetuximab is an epidermal growth factor receptor {EGFR) blocker that inhibits the growth and survival of tumor cells that overexpress EGFR. Cetuximab has synergism with irinotecan and its addition to irinotecan in patients with advanced disease resistant to irinotecan increases response rate from 10% when cetuximab is used alone to 22% with combination of cetuximab and irinotecan. The addition of bevacizumab to FL in patients with advanced colorectal cancer has been reported to increase the response rate from 17% to 40%. In patients who undergo resection of liver metastases from colorectal cancer, postoperative treatment with a combination of hepatic arterial infusion of floxuridine and IV fluorouracil improves the outcome at 2 yr.

CHRONIC Rx Follow-up is indicated with: • Physician visits with a focus on the clinical and disease-related history, di-

rected physical examination guided by this history, coordination of follow-up, and counseling every 3 to 6 mo for the first 3 yr, then decreased frequency thereafter for 2 yr • Colonoscopy yearly for the initial 2 yr, then every 3 yr • CEA level should be obtained baseline; if elevated, it can be used postoperatively as a measure of completeness of tumor resection or to monitor tumor recurrence; if used to monitor tumor recurrence, CEA should be obtained every 3 to 6 mo for up to 5 yr. The role of CEA for monitoring patients with resected colon cancer has been questioned because of the small number of cures attributed to CEA monitoring despite the substantial cost in dollars and physical and emotional stress associated with monitoring

• •

DISPOSITION • The 5-yr survival varies with the stage of the carcinoma: 1. Dukes’ A 5-yr survival, 80% 2. Dukes’ B 5-yr survival, 60% 3. Dukes’ C 5-yr survival, 20% 4. Dukes’ D 5-yr survival, 3% • Overall 5-yr disease-free survival is approximately 50% for colon cancer. • High-frequency microsatellite instability in colorectal cancer is independently predictive of a relatively favorable outcome and, in addition, reduces the likelihood of metastases. • In patients with Dukes’ C (stage III) colorectal cancer there is improved 5yr survival among women treated with adjuvant chemotherapy (53% with chemotherapy vs. 33% without) and among patients with right-sided tumors treated with adjuvant chemotherapy. • Retention of 18q alleles in microsatellite-stable cancers and mutation of the gene for the type I receptor for TGFB1 in cancers with high levels of microsatellite instability point to a favorable outcome after adjuvant chemotherapy with fluorouracil-based regimens for stage II colon cancer.

REFERRAL • Surgical referral for resection • Oncology referral for adjuvant chemotherapy in selected patients • Radiation oncology referral for patients with stage II or III rectal cancers

PEARLS & CONSIDERATIONS COMMENTS • Decreased fat intake to 30% of total energy intake, increased fiber, and fruit and vegetable consumption may lower colorectal cancer risk. Recent literature

•

•

•

reports, however, do not support a protective effect from dietary fiber against colorectal cancer in women. Chemoprophylaxis with aspirin (81 mg/day) reduces the incidence of colorectal adenomas in persons at risk. The National Cancer Institute has published consensus guidelines for universal screening for hereditary nonpolyposis colon cancer (HNPCC) in patients with newly diagnosed colorectal cancer. Tumors in mutation carriers of HNPCC typically exhibit microsatellite instability, a characteristic phenotype that is caused by expansions or contractions of short nucleotide repeat sequences. These guidelines (Bethesda Guidelines) are useful for selective patients for microsatellite instability testing. Screening patients with newly diagnosed colorectal cancer for HNPCC is cost effective, especially if the benefits to their immediate relatives are considered. Expression of guanylyl cyclase C mRNA in lymph nodes is associated with recurrence of colorectal cancer in patients with stage II disease. Analysis of guanylyl cyclase mRNA expression by RT-PCR may be useful for colorectal cancer staging. The use of either annual or biennial fecal occult-blood testing significantly reduces the incidence of colorectal cancer. In addition, screening should include a yearly rectal exam and a colonoscopy every 10 years. There is no evidence-based information to provide an upper age limit for screening. The detection of mutations in the adenomatous polyposis coli (APC) gene from stool samples is a promising new modality for early detection of colorectal neoplasms.

SUGGESTED READINGS Andre T et al: Oxaliplatin, fluorouracil, and leucovorin as adjuvant treatment for colon cancer, N Engl J Med 350:2343, 2004. Baron JA et al: A randomized trial of aspirin to prevent colorectal adenomas, N Engl J Med 348:891, 2003. Cunningham D et al: Cetuximab monotherapy and cetuximab plus irinotecan in irinotecanrefractory metastatic colon cancer, N Engl J Med 351:337, 2004. Hurwitz H et al: Bevacizumab plus irinotecan, fluorouracil, and leucovorin for metastatic colon cancer, N Engl J Med 350:2335, 2004. Mayer R: Two steps forward in the treatment of colorectal cancer, N Engl J Med 350:2406, 2004. Pfister D et al: Surveillance strategies after curative treatment of colorectal cancer, N Engl J Med 350:2375, 2004. AUTHOR: FRED F. FERRI, M.D.

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Conjunctivitis DISPOSITION

DEFINITION

• History and physical examination • Reports of itching, pain, visual changes

Follow carefully for the first 2 wk to make sure secondary complications do not occur.

LABORATORY TESTS

REFERRAL

Cultures are useful if not successfully treated with antibiotic medications; initial culture is usually not necessary.

To ophthalmologist if symptoms refractory to initial treatment

The term conjunctivitis refers to an inflammation of the conjunctiva resulting from a variety of causes, including allergies and bacterial, viral, and chlamydial infections.

SYNONYMS “Red eye”/“Pink eye” Acute conjunctivitis Subacute conjunctivitis Chronic conjunctivitis Purulent conjunctivitis Pseudomembranous conjunctivitis Papillary conjunctivitis Follicular conjunctivitis ICD-9CM CODES 372.30 Conjunctivitis, unspecified

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE (IN U.S.): • Very common • Often seasonal and can be extremely contagious PREDOMINANT AGE: Occurs at any age PEAK INCIDENCE: More common in the fall when viral infections and pollens increase

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Injection and chemosis of conjunctivae with discharge (Fig. 1-12) • Cornea clear • Vision often normal

ETIOLOGY • • • • •

Bacterial Viral Chlamydial Allergic Traumatic

TREATMENT

PEARLS & CONSIDERATIONS

NONPHARMACOLOGIC THERAPY

COMMENTS

• Warm compresses if infective conjunctivitis • Cold compresses in irritative or allergic conjunctivitis

ACUTE GENERAL Rx • Antibiotic drops (e.g., levofloxacin, ofloxin, ciprofloxacin, tobramycin, gentamicin ophthalmic solution one or two drops q2-4h) • Caution: be careful with corticosteroid treatment and avoid unless sure of diagnosis; corticosteroids can exacerbate infections • Povidine-iodine (Betadine) eyedrops when available

CHRONIC Rx • Depends on cause • If allergic, nonsteroidals such as Voltaren ophthalmic solution, mast cell stabilizers such as Alocril, Patanol, Zaditor are useful • If infectious, antibiotic drops (see Acute General Rx) • Dry eyes need artificial tears, ristasis, lacrimal duct plugs when indicated

• Red eyes are not just conjunctivitis when there is significant pain or loss of sight. However, it is usually safe to treat pain-free eyes and the normal seeing red eye with lid hygene and topical treatment. • Beware of patients wearing soft contact lenses and the elderly. • Do not use steroids indiscriminately; use only when the diagnosis is certain.

SUGGESTED READINGS Bennett C: Treatment of viral conjunctivitis in children, Am Fam Physician 67(9):1873, 2003. Fischer PR et al: Route of antibiotic administration for conjunctivitis, Pediatr Infect Dis J 21(10):989, 2002. Nichols GR: The red eye, N Engl J Med 343(21):1577, 2000. Sheikh A, Hurwitz B: Topical antibiotics for acute bacterial conjunctivitis: a systematic review, Br J Gen Pract 51:473, 2001. AUTHOR: MELVYN KOBY, M.D.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • •

Acute glaucoma Corneal lesions Acute iritis Episcleritis Scleritis Uveitis Blepharitis Canalicular obstruction The differential diagnosis of red eye is described in Section II FIGURE 1-12 Conjunctival infection from viral conjunctivitis. (From Marx JA [ed]: Rosen’s emergency medicine, ed 5, St Louis, 2002, Mosby.)

DISEASES AND DISORDERS

WORKUP

Section I

BASIC INFORMATION

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Constipation BASIC INFORMATION DEFINITION A recent consensus panel defined constipation as at least 12 weeks, not necessarily consecutive, within the previous 12 months of two or more of the following: straining, lumpy/hard stools, sensation of incomplete evacuation, sensation of anorectal obstruction, manual maneuvers to facilitate stooling, or less than three defecations per week. The patient should be devoid of loose stools and not meet criteria for irritable bowel syndrome. More commonly, constipation is present when a patient has two or less bowel movements per week or the patient complains of straining with defecation. ICD-9CM CODES 594.0

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE (IN U.S.): The literature estimates the prevalence to be between 2% and 28%. An inconsistent definition of constipation has resulted in the variation of these findings. PREDOMINANT SEX: Women PREDOMINANT AGE: Prevalence increases with age.

PHYSICAL FINDINGS & CLINICAL PRESENTATION On rectal exam the physician should evaluate for the presence of rectal tone, anorectal masses, stricture, fecal impaction, consistency of the rectal wall. A pelvic exam should be performed as pelvic floor dysfunction or a rectocele can lead to alteration in bowel movements.

ETIOLOGY Behavioral: • Inactivity, poor bowel habits are common causes of constipation in the older population. Medications: • Most commonly anticholinergics and opiates; however, other drugs including calcium channel blockers, iron supplements, chronic laxative abuse, NSAIDs, sucralfate, cholestyramine, and diuretics can lead to constipation.

Dietary: • Dehydration whether by decreased fluid consumption, aggressive diuresis, or other insensible fluid losses can result in constipation. No study has shown that low fiber intake results in constipation; yet, increasing a person’s fiber intake can effectively treat constipation. Structural: • Colonic lesions—stricture, mass, ulcer • Anorectal lesions—prolapse, stricture, fissure, ulcer, mass Systemic disease: • Endocrine—hypothyroidism, hyperparathyroidism, diabetes mellitus • Metabolic—hypokalemia, hypercalcemia, uremia, porphyria Neurologic disorders • Parkinson’s disease, multiple sclerosis, damage to the sacral nerve, autonomic dysfunction Pelvic floor dysfunction Irritable bowel syndrome

DIAGNOSIS WORKUP The first step is to identify that the patient meets the definition of constipation. Physicians should ascertain what is most distressing to the patient and request a journal of diet regimen and bowel patterns. The majority of elderly patients have constipation symptoms due to poor eating habits, immobility, and medications. A review of all medications, including over-the-counter medications, is imperative because alteration in bowel habits is a side effect of a variety of medications. All patients with new-onset constipation, thinning of stools, weight loss, hematochezia/melena should have an evaluation for abnormality of colonic or anorectal structures (e.g., rule out colon cancer).

LABORATORY TESTS A patient’s history will direct laboratory testing—potassium, calcium, blood glucose, TSH.

IMAGING STUDIES Anatomic lesions can be visualized with colonoscopy, barium enema, or virtual colonoscopy.

TREATMENT GENERAL Rx Interestingly, although immobility is a risk factor of constipation, exercise has not been shown to be an effective treatment for constipation.

PHARMACOLOGIC MANAGEMENT (TABLE 1-2) The patient should gradually increase fiber supplementation every 7 to 10 days and, if necessary, advance to saline agents like milk of magnesia or Epsom salts. A patient could alternatively be prescribed a stool softener. Patients can increase the dose of stool softener with the goal of attaining soft, not liquid, stools. If the previous measures fail, then the physician should add a stimulant or an osmotic agent.

REFERRAL A gastroenterologist should be consulted when a colonoscopy is appropriate, when the primary physician has not identified the culprit condition causing symptoms of constipation, or when fiber supplementation/laxatives/stool softener have failed to alleviate the constipation.

PEARLS & CONSIDERATIONS • Pelvic floor dysfunction and irritable bowel syndrome should be considered in patients in whom pharmacologic management fails to control the symptoms of constipation. • All patients on narcotics should also be on a combination drug regimen including a stimulant and stool softener.

SUGGESTED READINGS Locke III GR, Pemberton JH, Phillips SF: American Gastroenterological Association practice technical review on constipation, Gastroenterology 119:1161-1178, 2000. Rao SSC: Constipation: evaluation and treatment, Gastroenterol Clin North Am 32:659683, 2003. AUTHOR: MICHAEL P. GERARDO, D.O., M.P.H.

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Constipation TABLE 1-2

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Pharmacologic Management of Constipation

Magnesium hydroxide, magnesium sulfate Stimulants Bisacodyl Senna Cascara Lubricants Mineral oils Enemas Tap water Mineral oils Phosphate Soap suds

Onset of Action

Side Effects

12-24 hr

Bloating and flatulence

1-4 tsp daily 1 tsp twice daily 1 tsp twice daily 1-2 tablets twice daily 12-72 hr 100 mg twice daily Cramping, bloating, flatulence 15-16 mL three times daily 15-60 mL three times daily 17 gm in 8 oz liquid once or twice daily 15-30 mL twice daily

24-48 hr 24-48 hr 0.5-1 hr

5-15 mL twice daily 2-4 tabs bid 5-15 mL prn

6-8 hr 8-12 hr 8-12 hr

15-45 mL twice daily

1-6 hr

500 mL per rectum 100-250 mL per rectum 120 mL per rectum up to 1500 mL per rectum

5-15 min 1-6 hr 5-15 min 5-15 min

3-12 hr Cramping, bloating, flatulence

Malabsorption of fat-soluble vitamins Trauma, hyperphosphatemia

DISEASES AND DISORDERS

Fiber Bran Psyllium Methylcellulose Calcium polycarbophil Stool Softener Docusate sodium Osmotic Agents Sorbitol Lactulose Polyethylene glycol (PEG)

Dosage

Section I

Type

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Delirium BASIC INFORMATION DEFINITION Delirium is an acute change in mental status that is not otherwise explained by an underlying psychiatric illness such as dementia. It is often a harbinger of a serious medical illness in older persons and may be the only presenting sign of an occult illness.

PHYSICAL FINDINGS & CLINICAL PRESENTATION

SYNONYMS Acute change in mental status Acute confusion Acute brain failure Delirium tremens—associated alcohol withdrawal

• Common precipitating risk factors include:  Medications (narcotics or sedatives)  Immobilization  Restraints  Dehydration  Indwelling catheters  Infection  Metabolic abnormalities (e.g., hypoxia, hypoglycemia)

with

ICD-9CM CODES 290.11 Presenile dementia with delirium 290.3 Senile dementia with delirium 290.41 Multi-infarct dementia with delirium 291.0 Alcohol withdrawal delirium 292.81 Drug-induced delirium 293.0 Acute delirium 293.1 Subacute delirium 293.81 Delirium, transient organic with delusions 293.82 Delirium, transient organic with hallucinations 296.0 Mania with delirium

EPIDEMIOLOGY & DEMOGRAPHICS Delirium may arise at any point during the course of a medical illness but is most common in the hospital setting. Prevalence rates range from 10% to 40% on admission to the hospital with another 25% to 50% developing delirium during the course of hospitalization. Postoperative and intensive care patients are at much greater risk of delirium with rates of 10% to 60% and 70% to 87%, respectively. There is no gender difference with delirium. • Delirium is a multifactorial syndrome and is thus caused by multiple interrelated factors. Essentially any medical condition may precipitate a delirium but older persons have identifiable risk factors that increase their risk of delirium and contribute to the development and severity of delirium. These are often divided into baseline or predisposing risk factors and hospital-related or predisposing risk factors. • Common predisposing risk factors are:  Medications (anticholinergic medications)  Comorbid conditions  Sensory impairment  Malnutrition  Preexisting cognitive impairment

• Delirium must occur in the context of a medical illness, metabolic derangement, drug toxicity, or drug withdrawal. The symptoms cannot be explained by an underlying dementia. • Symptoms and signs:  Acute change in mental status  Fluctuating course  Inattention  Disorganized thinking  Altered level of consciousness (hypoactive/lethargic or hypervigilant and agitated)  Altered perceptions (illusions, hallucinations)  Decreased function • Review history, establish baseline cognitive and functional status, and look for symptoms of occult infection/illness. • Review medications (new, omitted, withdrawn, interactions, herbal therapies, OTC). • Full set of vital signs. • Complete physical exam. • Neurologic exam (new focal deficits).

ETIOLOGY The etiology of delirium is unknown. Increasing evidence points to the suppression of the cholinergic nervous system as a strong contributor.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Dementia • Depression • Acute psychotic illness

WORKUP The initial diagnosis of delirium must include a thorough assessment of baseline cognitive function in older persons, especially for those with potential preexisting cognitive impairment. Often, caregivers and family members can provide valuable information concerning baseline functional and cognitive status. According to the Confusion Assessment Method (CAM), a screening test for detecting delirium, the diagnosis of delirium is made if the following have been met: 1. There is an acute change in cognition based on an objective cognitive assessment and a fluctuating course.

2. Inattention is present; that is, difficulty focusing on the task at hand or easily distracted. 3. Disorganized thinking is present, including decreased memory, disorientation, altered visual perceptions. 4. Altered level of consciousness is present, which can be present as either agitation or hypervigilance, or lethargy and hypoactive state. Criteria 1 and 2 must be present and either 3 or 4 to screen positive for delirium.

LABORATORY TESTS • No specific test exists to screen for or diagnose delirium. Refer to algorithm for details. • Complete blood count, electrolytes including calcium, glucose, kidney function, urinalysis, urine culture. • Consider, blood culture, TSH, ABG, cortisol, vitamin B12 level, toxic screen, drug levels if applicable, or lumbar puncture with CSF analysis (for fever of unknown origin).

IMAGING STUDIES • CXR to rule out occult pneumonia, CHF • Brain imaging—with focal neurologic signs and symptoms

TREATMENT NONPHARMACOLOGIC THERAPY Treatment involves both the identification and reduction of any potential risk factors and the treatment of acute agitation simultaneously. Identification of risk factors: • It is important to identify all contributing risk factors and try to eliminate or reduce any risk factors that can be changed. For example, reorientation, assistance with nutrition and fluid intake, or elimination of medications with deliriogenic or anticholinergic properties. Removal of restraints and prevention of bed rest with frequent ambulation in the hallway will prevent delirium and help reduce symptoms. • Other nonpharmacologic strategies include increasing the family presence to reduce anxiety, transferring to a quiet room, and correcting sensory deficits (using glasses for vision loss and hearing amplifier devices for hearing loss). • Use of a nonpharmacologic sleep protocol (which includes a backrub, warm drink, relaxing music, and uninterrupted sleep) has been validated as an important method to avoid sleep medications and prevent delirium.

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Delirium ACUTE GENERAL Rx

• Minimize medications that can contribute to delirium. • Minimize risk factors. • Avoid hospitalization if possible. • Discontinue or wean off antipsychotic or anxiolytic medications that were begun for acute management of agita-

DISPOSITION • Older persons with delirium are at higher risk of dying or being transferred to a skilled nursing facility where the symptoms of delirium can persist for up to 6 months. Delirium is also associated with an increased hospital and postacute cost of care.

REFERRAL • Consider referral to a geriatrician or geriatric psychiatrist for complicated delirium or for patients with agitation that is difficult to manage.

PEARLS & CONSIDERATIONS COMMENTS • Delirium is common in the elderly and associated with negative outcomes. • Delirium is often the only sign of a life-threatening medical illness. • Delirium is a multifactorial syndrome often precipitated by multiple predisposing and precipitating risk factors. • Older persons with dementia are at much higher risk of developing delirium and are more likely not to be recognized as having a delirium. • Recognition of delirium can be enhanced by using tools such as the Confusion Assessment Method.

• Treatment of delirium should include both the reduction of risk factors and the treatment of agitation.

PREVENTION • Identification of high-risk patients and implementing nonpharmacologic strategies can help reduce the rate of delirium by 33%. Avoidance of medications highly associated with delirium can also prevent the development of delirium.

PATIENT/FAMILY EDUCATION • Patients and families should be educated on delirium if it occurs to improve understanding of the changes and that most of the changes are reversible.

SUGGESTED READINGS Hospital Elder Life Program: http://elderlife.med.yale.edu/public/public-main.php Inouye SK et al: A multicomponent intervention to prevent delirium in hospitalized older patients, New Engl J Med 340:669, 1999. Marcantonio ER et al: Reducing delirium after hip fracture: a randomized trial, J Am Geriatr Soc 49:516, 2001. McCusker J et al: Delirium predicts 12-month mortality, Arch Intern Med 162:457, 2002. McDowell JA et al: A non-pharmacological sleep protocol for hospitalised older persons, J Am Geriatr Soc 46:700, 1998. AUTHOR: LYNN MCNICOLL, M.D.

DISEASES AND DISORDERS

CHRONIC Rx

tion and delirium. These medications are often continued indefinitely for no definite reason and result in significant side effects.

Section I

• Medications to treat delirium should only be used in severely agitated individuals whose behavior threatens to interrupt medical care or if a safety hazard for patients or staff exists. • Doses should be started at the lowest dose and for a limited time. Often standing low doses of antipsychotics may help manage symptoms with minimal side effects. Recommendations are largely based on empiric evidence; little research on treatment of delirium exists. • Types of medications:  Haloperidol 0.25 to 1.0 mg may be repeated every 30 minutes until the patient is calm and alert. Maximum 24-hour dose should be limited to 5 mg.  Atypical antipsychotics including risperidone, olanzapine, and quetiapine can also be used with the same principles.  Benzodiazepines, especially lorazepam, are the drugs of choice for alcohol or sedative withdrawal but should be limited to third line therapy for agitation.

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Dementia BASIC INFORMATION DEFINITION Dementia means loss of mental functions. It is a syndrome of acquired and persistent cognitive deficits that includes memory impairment in association with impairments in abstract thinking, judgment, or other disturbances of higher cortical function (e.g., aphasia, apraxia, agnosia, executive functioning) or personality change. The disturbances are severe enough to interfere significantly with work, usual social activities, or relationships with others. Dementia is not diagnosed if the symptoms occur exclusively in the context of delirium. It must be an acquired loss of function, and hence is distinguished from developmental disorders such as mental retardation. Dementia can have many etiologies, as will be noted. (Adapted from DSM-III-R and DSM-IV.) ICD-9CM CODES 290.0 Senile dementia, uncomplicated DSM-IV: Dementia of the Alzheimer’s type, late onset 290.1 Presenile dementia DSM-IV: Dementia of the Alzheimer’s type, early onset, uncomplicated 290.4 Arteriosclerotic dementia DSM-IV: Vascular dementia 294.1 Dementia in conditions classified elsewhere (code first underlying physical condition, e.g., listed as follows) DSM-IV: Dementia due to: 331.0 Alzheimer’s disease 331.82 Dementia with Lewy bodies 331.19 Frontotemporal dementia 310.1 Mild memory disturbances, not amounting to dementia, associated with senile brain disease 294.9 Unspecified organic brain syndrome (chronic) DSM-IV: Cognitive Disorder NOS

SYNONYMS Senile dementia Presenile dementia Senile psychosis

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: Annual dementia incidence rate approximately doubles every 5 years after age 65; approximately 7 to 9 per 1000 at age 65-69 and 85 to 118 per 1000 at age 85 and older. PREVALENCE: Approximately 8% to 10% of U.S. population (4 million to 6 million) above age 65 have dementia; prevalence approximately doubles every 5 years after age 65; prevalence is ap-

proximately 2% at age 65 and 34% to 68% at age 85 and older. PREDOMINANT ETIOLOGY: Alzheimer’s disease (AD) is by far the most common cause of dementia. Approximately 55% to 70% of all dementias have a significant contribution from Alzheimer’s pathology and approximately 35% of all dementias may be attributable to pure AD. Vascular dementia and dementia with Lewy bodies are thought to be the next most common etiologies. Prevalence rates of dementia etiologies vary across ethnic groups. PREDOMINANT SEX: Varies by dementia diagnosis; AD is more common in females; vascular dementia and dementia with Lewy bodies is more common in males; frontotemporal dementia appears to occur equally in males and females. PREDOMINANT AGE: Incidence and prevalence increase over age 65 and are highest at age 85 and older; average age of onset is younger in frontotemporal dementia and some relatively rare, familial subtypes of AD (see as follows). PEAK INCIDENCE AND PREVALENCE: Age 85 and older. GENETICS: First-degree relatives may be at increased risk for certain types of dementia (e.g., AD, frontotemporal dementia); apolipoprotein 4 allele is a genetic risk factor for AD but is not diagnostic. Genetic disorders causing dementia include Huntington’s disease, CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), and early-onset familial AD. Some forms of frontotemporal dementia have genetic linkages.

PHYSICAL FINDINGS & CLINICAL PRESENTATION See Fig. 1-13. Physical findings and clinical presentation differ across dementia etiologies.

ETIOLOGY ALZHEIMER’S DISEASE Prevalence: AD is the most common cause of dementia; contributes to dementia in approximately 55% to 70% of all cases; pure AD accounts for approximately 35% of all cases. Core features: • Gradually progressive memory impairment with insidious onset. • Impairment in executive function, spatial abilities, and language follow later. • Behavioral dysregulation may also occur later. Pathology: • Extraneuronal plaques (protein:  amyloid-42). • Intraneuronal neurofibrillary tangles (protein: hyperphosphorylated form of tau). • Definitive diagnosis requires clinicopathologic correlation.

Genetics and family history: • Risk of AD is about four times greater in patients with a first-degree relative with AD. • ApoE 4 allele on chromosome 19 increases risk of AD and shifts age of onset 3 to 7 years earlier than in those with sporadic onset who do not have an 4 allele; risk is further increased in presence of first-degree relative with AD. • Approximately 2% of AD cases are early-onset familial AD related to mutations in either chromosome 14 (presenilin 1), 1 (presenilin 2), or 21 (amyloid precursor protein). • Almost all patients with Down syndrome will develop AD pathology after age 40. Key cognitive features: • Memory impairment is cardinal feature; impairment in language, visuoconstructional ability, executive functioning, and other cognitive domains follows. • Motor and sensory function generally intact until late stages. Neurologic features: • Normal exam early. • Later stages can be associated with extrapyramidal or parkinsonian features. • Impairment in gait, speech, urinary and fecal continence, and reemergence of primitive reflexes may occur in final stages. Key neuroimaging features: • Useful for ruling out alternate (e.g., neoplasm, hydrocephalus) or concomitant (e.g., vascular lesions) causes of dementia. • MRI may reveal diffuse cortical atrophy with ex vacuo ventricular enlargement particularly in temporal horns of lateral ventricle due to hippocampal volume loss; severity of imaging findings generally increases with severity of dementia. • Temporoparietal hypometabolism on SPECT or PET. • Neuroimaging is not diagnostic. Course: • Insidious onset with gradual progression; memory is usually first symptom. • Average survival is about 8 years depending on age at diagnosis and concomitant medical conditions; survival may be up to 20 years. VASCULAR DEMENTIA (VAD) Prevalence: VaD is the second or third most common cause of dementia, accounting for about 10% to 30% of all dementia cases; prevalence increases with age; more common in men. Core features: • Focal neurologic signs/symptoms. • Neuroimaging evidence of cerebrovascular disease.

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Cognitive complaint/symptom

Section I

Objective cognitive/behavioral dysfunction

Within normal limits

Mild deficits, ADLs generally intact Normal aging Age-associated cognitive decline Possible MCI

At least mild deficits in memory and other cognitive domains, clear impact on ADLs

• Impairment in executive functioning, speed of mental processing, with relatively mild memory deficits in most cases (particularly true for VaD due to microvascular disease). • Apathy may be prominent. Pathology: VaD can result from largevessel or small-vessel disease or a combination of the two. Most VaD cases are caused by small-vessel disease. Mendez and Cummings (2003) identify three main pathologic mechanisms of VaD: • Large-vessel (macrovascular) VaD typically arises from multiple bilateral thromboembolic infarctions; involvement of cortical regions. • Small-vessel (microvascular) VaD typically arises from arteriosclerotic changes in subcortical arteries causing injury to periventricular and deep white matter and to subcortical grey nuclei (e.g., caudate, putamen, and thalamus). The injuries include complete infarction (i.e., lacunar infarction) and incomplete infarction that appear as diffuse white matter lesions on neuroimaging (i.e., hyperintense on T2 MRI sequences; hypodense on CT). In some cases, a single strategic small-vessel infarction in a critical circuit (e.g., left thalamus) may cause multiple cognitive deficits of sufficient

Neuropsychology Psychiatric Other explanations

Possible dementia

Neuropsychological assessment for diagnosis, etiology, baseline for future comparison FIGURE 1-13

Equivocal

Specific diagnosis/probable etiology

Evaluation of dementia. ADLs, Activities of daily living

severity to warrant a diagnosis of dementia. Diffuse white matter lesions may also cause dementia, if sufficiently extensive (i.e., involves 25% of white matter). • Episodes of hypoperfusion hypoxia. Genetics and family history: • Increased risk with family history of stroke. • Genetic causes are relatively rare; CADASIL is one cause of dementia due to microvascular disease; it is autosomal dominant and linked to mutation in the notch3 gene on chromosome 19. Key cognitive features: • When due to multiple cortical infarcts, the pattern of cognitive impairment may vary depending on location of infarcts (e.g., language deficits from left hemisphere lesion, spatial deficits from right hemisphere lesion). • Subcortical ischemic vascular disease is characterized by deficits in psychomotor processing speed, mental flexibility, executive function, and memory retrieval. • Single subcortical strategic infarct (i.e., anterior thalamus) may be sufficient to cause widespread cognitive dysfunction and dementia. • Apathy may be prominent.

Neurologic features: • Focal neurologic signs or symptoms (e.g., exaggerated deep tendon reflexes, extensor plantar response, spastic limb weakness) that vary with location and size of lesions. Key neuroimaging features: • Evidence of cortical or subcortical infarction. • Diffuse areas of high signal on T2weighted MRI (low signal on CT), particularly in periventricular and deep white matter regions. • Clinical or radiologic evidence of cerebrovascular disease required for diagnosis. • Absence of cortical or hippocampal atrophy adds confidence to differential diagnosis of VaD vs. AD. Course: • Difficult to predict. • May have abrupt onset with stepwise course. • Onset and course may be more gradual, especially when due to subcortical small-vessel disease. • Onset and progression may be gradual with punctuated declines associated with discrete cerebrovascular events. • High mortality rate and lower life expectancy than AD; 50% survival rate from symptom onset is about 6.7

DISEASES AND DISORDERS

Clinical hx Physical and neuro exam MSA and behavior assmt Labs and imaging Reversible causes

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Dementia years; heart disease or stroke is common cause of death. DEMENTIA WITH LEWY BODIES (DLB) Prevalence: Second or third most common cause of dementia; accounts for approximately 14% to 20% of all dementia cases. • Almost two thirds are male. • Majority do not have pure DLB but also have concomitant AD pathology. Core features: • Parkinsonism • Cognitive fluctuations • Visual hallucinations (occur in 40% to 70% of cases)  Usually vivid, well-formed of humans or animals • Dementia plus two core features are needed for diagnosis of probable DLB; one core feature is needed for possible DLB. • Other supportive features:  Repeated falls  Syncope; transient loss of consciousness  Delusions  Rapid eye movement (REM) sleep behavior disorder  Neuroleptic sensitivity Pathology: Lewy body inclusions (protein: mainly -synuclein, but ubiquitin and tau are also present) in diffuse brain regions including cortical neurons. Genetics and family history: • No genes identified in sporadic DLB cases. • A rare autosomal dominant form exists related to mutations in -synuclein gene on chromosome 4. Key cognitive features: • Marked attentional fluctuations. • Frontal-executive dysfunction. • Prominent visuospatial impairment. • Deficits occur in memory, confrontational naming, and praxis; may not be as severe as in AD. Neurologic features: • Extrapyramidal signs present in 25% to 50% at diagnosis and most develop it over disease course. • Up to 25% of autopsy-confirmed cases may not have report of extrapyramidal symptoms. • Axial bias to symptoms; i.e., greater postural instability, gait impairment, and facial impassivity and relatively less tremor than in Parkinson’s disease. Key neuroimaging features: • Less pronounced hippocampal/medial temporal lobe atrophy compared with AD. • Quantitative volumetric measurements may show atrophy of putamen. • Neuroimaging not diagnostic. Course: • Initial symptoms could be parkinsonism followed by cognitive impairment or the opposite: cognitive symptoms followed by parkinsonism.

• Shorter survival times than AD; about 7.7  3.0 years from onset of cognitive symptoms. FRONTOTEMPORAL DEMENTIA (FTD) Prevalence: • Accounts for approximately 2% to 5% of all dementia cases. • Accounts for 20% of neurodegenerative dementias in people under 65 years old. • Average age of onset is 57 years with a typical range of 51 to 63. Core features: • Personality changes are most prominent symptom and typically precede cognitive changes:  Decline in social comportment  Impaired regulation of personal conduct  May become disinhibited but are more likely to display apathy  Lack of attention to personal hygiene can be supportive of diagnosis Pathology: • FTD is the main syndrome of frontotemporal lobar degeneration (FTLD)—a spectrum of dementing disorders involving degeneration of the frontal and anterior temporal lobes. • Mendez and Cummings (2003) describe three main pathologic variants of FTD:  Lacking distinctive histology (FTLDldh): neuronal loss, astrogliosis with spongiosis in frontotemporal cortex  Pick bodies (FTLD-Pick): severe frontal atrophy (“knife-like” gyri); enlarged neurons called Pick cells and neuronal Pick body inclusions (tau-positive, ubiquitin-positive); FTLD-Pick is diagnosed when Pick bodies are present irrespective of presence of Pick cells  Motor neuron disease (MND): involvement of anterior horn cells with or without ubiquitin inclusions Genetics and family history: • 38% to 50% of cases have positive history of similar dementia in first-degree family member. • Several mutations in tau gene have been found in familial FTD. • Some cases associated with mutations on chromosome 17; linkage to chromosome 3 in some families; presenilin 1 may be involved in some phenotypes. Key cognitive and behavioral features: • Early blunting of emotional responsiveness; loss of basic emotions. • Executive cognitive impairment prominent. • Compulsive behaviors are common:  Klüver-Bucy syndrome may occur: emotional blunting, reduction or loss of fear responding, oral explo-

ration of the environment with dietary preference alterations, hypermetamorphosis (exploration of environmental stimuli as soon as noticed), sensory agnosia, altered sexual behavior. • Reduction of verbal output. • Pattern of memory deficits suggests retrieval impairment rather than storage impairment characteristic of AD. Neurologic features: • Neurologic features may be absent early with exception of primitive (“frontal release”) reflexes (grasp, snout, suck). • Parkinsonism may be present; may precede dementia and may resemble progressive supranuclear gaze palsy; fasciculations may occur. • Urinary or fecal incontinence may occur later. Key imaging features: • MRI might not be sensitive to early changes; the majority of patients will eventually show anterior-posterior asymmetry of cortical atrophy, being greater in frontal and anterior temporal lobe regions than in posterior brain regions. • SPECT reveals frontotemporal hypometabolism • Neuroimaging not diagnostic by itself. Course: • Initial and most prominent symptoms are usually changes in personality, social awareness, and behavior, especially disinhibition or apathy. • Mean age of onset is 57 years with a range of 51 to 63 years. • Duration is typically 8 to 11 years; may be shorter in MND variant. MISCELLANEOUS CAUSES OF DEMENTIA Toxic-metabolic etiologies (4%, including alcohol, recreational drugs, medications); infectious (3%); other movement disorders (6%, e.g., Parkinson’s disease, progressive supranuclear palsy, Huntington’s disease, corticobasal degeneration); normal pressure hydrocephalus (2.5%); other psychiatric (4%); and miscellaneous (1%, e.g., dementia due to AIDS, head trauma) POTENTIALLY REVERSIBLE CAUSES OF DEMENTIA Medication toxicity/drug interactions; hydrocephalus; brain tumor; infection; electrolyte imbalance; malnutrition; metabolic (i.e., B12 deficiency, hepatic encephalopathy); and endocrine disorders (i.e., hypothyroidism) COGNITIVE CHANGES IN NORMAL AGING • General slowing of mentation and physical responses; declines occur in complex and divided attention, ability to recall names, executive and visuospatial functions; reduced efficiency in learning and spontaneous recall of

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Dementia

• Two-step process: 1. Is dementia present? and if so 2. What is the most likely etiology? • No definitive test or algorithm for determining the presence or absence of dementia exists; differentiating normal aging from MCI and MCI from mild dementia is difficult.

DIFFERENTIAL DIAGNOSIS • Determining etiology requires careful assessment of cognitive and behavioral symptoms, neurologic features, and the onset and temporal course of illness along with laboratory and neuroimaging assessments. • Determining etiology requires differentiating non-AD dementia from AD. DEMENTIA VS. AMNESIA • Amnesia is isolated and profound memory impairment; dementia involves multiple cognitive domains. DEMENTIA VS. APHASIA • Difficult because many cognitive tests require a strong language component. • Nonverbal testing may reveal intact non-language-based cognitive functions.

WORKUP CLINICAL HISTORY • Key symptoms; onset and progression; impact on activities of daily living. • Obtain information from a reliable informant. • Obtain a medication history and identify those that can disrupt cognition. • Screen for depression. • Family history of dementia. • Review of systems. PHYSICAL AND NEUROLOGIC EXAM • General health • Focal neurologic signs

• Frontal release signs • Apraxias MENTAL STATUS ASSESSMENT (MSA) • Evaluate arousal, orientation, attention/mental control, memory, language, conceptual or semantic knowledge, praxis (ability to perform skilled movements, e.g., ask the patient to pretend to light a cigarette), mental calculations, visuoconstructional abilities, executive functions, and behavior. • Mini Mental State Exam (MMSE): the most commonly administered formal mental status test; takes 5 to 10 minutes (see Appendix 2); can be supplemented with assessment of executive functions and abstract reasoning.  Executive function 1. Alternating figures: copy and continue producing alternating figures (e.g., O O). Look for loss of sequence or perseverations. 2. Alternating movements: place both hands on a table, one in a fist, one palm down; then alternate repeatedly. Demonstrate briefly and then have the patient continue independently. Look for loss of sequence.  Abstract conceptualization: similarities (e.g., “How are an apple and a banana alike?” include more than one similarity pair e.g., boat-car, watch-ruler, desk-bookcase). Look for concrete responses; differences instead of similarities. Responses can be influenced by educational and intellectual background. • Mini-Cog exam consists of a 3-item memory test and clock drawing; it takes 2 to 3 minutes to administer. It may be as accurate as the MMSE in a primary care setting. • Behavior: appearance; mood and affect; level of insight; thought content and process; psychosis; anxiety disorders; sleep and appetite.

LABORATORY TESTS • Blood  Thyroid panel or thyroid stimulating hormone  Complete blood count, vitamin B12, folate  Electrolytes, BUN, creatinine  Liver function tests  Glucose  Syphilis or AIDS if high clinical suspicion  Other tests directed by differential diagnosis • Urinalysis • Lumbar puncture: optional; if suspicion of cancer, infectious process, atypical presentation (e.g., rapid progression) • EEG: if history of seizures, rapid decline, suspicion of Creutzfeldt-Jacob disease

DISEASES AND DISORDERS

DIAGNOSIS

• Patients with aphasia are more likely than dementia patients to recognize and respond to what is happening around them; and to remember people, places, and routines. DEMENTIA VS. DEPRESSION • Depression with cognitive impairment is common in the elderly and may be a risk factor for dementia. Moreover, depression occurs in 12% to 20% of patients with dementia. • Cognitive deficits: attention, psychomotor processing speed, executive functioning, and memory retrieval; may reflect reduced concentration, loss of interest, and motivation. • Neuropsychologic testing may be helpful. Depressed patients are likely to benefit from cueing and recognition formats during memory testing, whereas AD patients do not. • Follow-up assessment after a period of treatment may be needed to rule out dementia. DEMENTIA VS. DELIRIUM • Delirium generally has a more rapid onset than dementia. • Marked attentional disturbances/ fluctuations are more common in delirium. • Delirium can be superimposed on a preexisting dementia or mild cognitive impairment. NORMAL AGING VS. MCI • The key judgments are whether the complaints and severity of cognitive impairment represent a decline that exceeds normal aging; the extent to which the impairments disrupt normal functioning; and whether they are better explained by psychiatric disorder, substance abuse, medication effects, situational stressors, or other nonneurodegenerative etiologies. • Neuropsychologic testing may be very helpful. MCI VS. MILD DEMENTIA • The key judgments are the scope and severity and stability of cognitive or behavioral impairment and the degree to which they affect daily functioning. • Neuropsychologic testing may be very helpful.

Section I

new information but memory storage is maintained. • Terms used to describe cognitive changes in aging: normal aging, ageassociated cognitive decline, age-associated memory impairment. MILD COGNITIVE IMPAIRMENT (MCI) • MCI is a syndrome (not an etiology) defined by subjective and objective impairment in memory or other cognitive domains that represents a decline and is greater than expected for age, but is of insufficient severity to significantly affect activities of daily living or to warrant a diagnosis of dementia. • The term mild cognitive impairment is often used to describe this period, but the term is not universally accepted and alternatives have been proposed (e.g., cognitive impairment no dementia). • Diagnostic criteria for MCI were developed based on those at high risk for AD. Criteria include subjective memory complaint, objective memory impairment for age/education on formal testing; generally intact global cognitive functioning; generally persevered daily functioning; and no dementia.  These criteria are now thought to describe amnestic MCI—a group with an approximately 12% annual rate of conversion to AD (vs. 1% to 2% for normal elderly).  Current MCI conceptualization includes other subtypes that might go on to develop different types of dementia.

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Dementia • Neuroimaging: CT or MRI of the head is appropriate to rule out hydrocephalus, mass lesions, and subdural hematomas and to assess for extent of cortical and subcortical atrophy, and cerebrovascular lesion load; SPECT or PET for cerebral metabolism may be helpful, especially for FTD

TREATMENT NONPHARMACOLOGIC THERAPY • Ensure patient safety: e.g., driving, wandering, cooking, ironing, tool use, medication management, nutrition, hygiene and grooming, financial management. • Establish routines, avoid chaotic or overstimulating environments. • Family education. • Caregiver support/respite: support groups, daycare, sharing caregiving with other family members. • Cerebrovascular risk factor control includes adequate treatment of hypertension, hypercholesterolemia, and diabetes.

PHARMACOLOGIC THERAPY • Cognitive impairment:  Acetylcholinesterase inhibitors (i.e., donepezil, galantamine, rivastigmine) approved for AD; they slow the course of cognitive decline, not disease modifying. May also be useful for vascular dementia.  Memantine (NMDA receptor antagonist) for moderate to severe AD.  For dementias other than AD, there are few pharmacologic options. • Agitation and psychosis:  Atypical antipsychotics including risperidone and olanzapine are widely used but not FDA approved.

Use lowest dose needed and try to wean off as soon as possible. Potential increased risk of cerebrovascular accidents. Avoid use of antipsychotics in patients with diffuse Lewy body dementia.  Benzodiazepines can occasionally be useful usually as second line therapy. • Depression: selective serotonin reuptake inhibitors can be effective for the treatment of coexisting depression among those with dementia.

• Reduce/control risk factors for cognitive impairment: cerebrovascular risk; medications that may contribute to cognitive impairment; alcohol; depression.

DISPOSITION

PATIENT/FAMILY EDUCATION

• Deterioration over time is a common feature of most dementias. • Goals of currently available treatments are to slow course and maximize functioning. • Transition to assisted living facilities may be helpful as dementia worsens; nursing home placement may follow.

• Alzheimer’s Association local support groups can offer useful education for families and caregivers.

REFERRAL • Neuropsychologic

assessment can help determine presence/absence of dementia and the pattern of cognitive impairment may help identify etiology. • Geriatric psychiatry if depression or other psychiatric disturbance is prominent. • Neurology referral is necessary to assess general neurologic status, especially if motor, sensory, or gait disturbance is present.

PEARLS & CONSIDERATIONS • Search for and treat potentially reversible causes of dementia. • Treat psychiatric disturbances (e.g., depression, agitation, psychosis, but be careful with antipsychotics in DLB).

PREVENTION • Reduction of cardiovascular risk factors including treatment of hypertension, hypercholesterolemia, and diabetes. • Promoting cognitive exercises and mental activity can help ward off progression. • Maintaining a healthy social life.

SUGGESTED READINGS Knopman DS et al: Practice parameter: diagnosis of dementia (an evidence-based review). Report of the quality standards subcommittee of the American Academy of Neurology, Neurology 56:1143-1153, 2001. McKeith I et al: Dementia with Lewy bodies, Lancet Neurol 3:19-28, 2004. Mendez MF, Cummings JL: Dementia: A Clinical Approach, ed 3, Philadelphia, 2003, Butterworth Heinemann. Petersen RC: Mild cognitive impairment as a diagnostic entity, J Intern Med 256:183-194, 2004. Roman GC: Vascular dementia revisited: diagnosis, pathogenesis, treatment, and prevention, Med Clin North Am 86:477-499, 2002. Rosenstein LD: Differential diagnosis of the major progressive dementias and depression in middle and late adulthood: a summary of the literature of the early 1990s, Neuropsychol Rev 8:109-167, 1998. Strub RL, Black FW: The Mental Status Examination in Neurology, ed 4, Philadelphia, 2000, FA Davis. AUTHORS: STEPHEN CORREIA, PH.D., and LYNN MCNICOLL, M.D.

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Depressive Disorder, Major BASIC INFORMATION

RELATED DISORDERS • Bipolar disorder includes MDEs and manic episodes. • Minor depressive disorder is defined as 2 weeks of two to four of the MDE criteria. • Dysthymic disorder is a low-grade chronic condition that entails depressed mood most days for 2 years without ever experiencing an MDE. • Psychotic depression is a severe form of MDD in which the criteria for an MDE are met and delusions or hallucinations are also present. • There are additional mood and anxiety disorders that include symptoms of depression. • In the context of bereavement or reactions to stress, consider the diagnosis of MDD if significant symptoms exist or if social or occupational functioning is impaired. • In the elderly, “depression” may encompass more than just MDD because some people experience severe depressive symptoms that do not meet diagnostic criteria for MDD. ICD-9CM CODES 296.2 Major depressive disorder, single episode 296.3 Major depressive disorder, recurrent episode 296.1 Manic disorder, recurrent episode 298.0 Depressive type psychosis 300.4 Neurotic depression 311 Depressive disorder, not elsewhere classified

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Studies suggest that the point prevalence of MDD in elderly community-dwelling individuals is about 1%. In addition, 3% to 4% of community elderly report symptoms consistent with dysthymia and 8% to 15% have clinically significant depressive symptoms. Among

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• Mental Status Examination, including assessment of appearance, alertness, orientation, speech/language function, reported mood, observed affect, thought content (with particular attention to the presence or absence of (1) suicidal thoughts, (2) homicidal thoughts, or (3) delusions), thought process (i.e., logical vs. disorganized), perception (if auditory hallucinations exist, check to see if the “voices” command or instruct the patient, and what the specific instructions entail), attention, memory, insight, and judgment. • Consider using the Geriatric Depression Scale (GDS) to quantify the intensity of depressive symptoms; and if significant concentration or attention disturbance exists, conduct a structured cognitive assessment such as the Mini Mental State Examination (MMSE). See Appendix 2 for scales. • If allowable, elicit additional behavioral information from family or wellinformed others (such as nursing home staff). • Risk assessment (see Pearls & Considerations).

• Older depressed patients sometimes focus on somatic complaints. • Any symptoms of an MDE could be the chief complaint, including paranoid thinking. • Carefully distinguish between psychomotor slowing and parkinsonism. • Additional symptoms include anhedonia, guilt feelings, frequent crying episodes, and a sense of helplessness and worthlessness. • Severe concentration disturbance in a depressed older person can mimic early dementia. • History of the depressive illness should include the following:  Past history of psychiatric symptoms (i.e., MDEs, manic symptoms, psychotic symptoms, substance abuse, dangerous behavior or emergency evaluation or inpatient treatment)  Targeted review of medical symptoms (i.e., fever, fatigue, weight loss, decreased exercise tolerance)  Assessment of situational factors (i.e., social support, financial strain, recent losses) • Physical examination should include a complete neurologic examination.

ETIOLOGY • Depression is likely the result of complex interactions between genetic predisposition, neurochemical and endocrine status, stressful psychosocial circumstances, and dysfunctional patterns of thinking. • Some depressive syndromes in late life may be due, in part, to frontostriatal and limbic system dysfunction. • Stroke is a risk factor for depression. • Subcortical atrophy may correlate with depressive symptoms in late life.

DIFFERENTIAL DIAGNOSIS • • • • • • • • •

Hypothyroidism Anemia Vascular disease Major organ system impairment (i.e., lymphoma, congestive heart failure, renal failure) Treatment side effect (i.e., antihypertensives, chemotherapy) Parkinson’s disease Dementia Consider other late-life mood or anxiety disorders Consider a primary sleep disorder if insomnia is prominent

WORKUP

LABORATORY TESTS • Thyroid panel or thyroid stimulating hormone • Complete blood count, vitamin B12, and folate • Electrolytes, blood urea nitrogen, creatinine • Additional tests as directed by the differential diagnosis

NEUROIMAGING & OTHER STUDIES • Neuropsychologic testing can help distinguish depression from dementia. • If significantly disrupted sleep or signs and symptoms of apnea are present, obtain a formal sleep study including electroencephalogram. • Brain MRI may be helpful in cases of known vascular disease.

DISEASES AND DISORDERS

Major depressive disorder (MDD) is defined as having one or more major depressive episodes (MDE). According to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), an MDE is defined as at least 2 weeks of depressed mood or loss of interest, accompanied by four or more of the following symptoms: change in appetite or weight, sleep disturbance, psychomotor agitation or slowing, fatigue or loss of energy, worthlessness or guilt, poor concentration or indecisiveness, and thoughts of suicide or death.

DIAGNOSIS Section I

DEFINITION

nursing home residents, major depression is present in 10% to 15% and depressive symptoms in another 18%. Among persons with dementia, depression rates can range from 15% to 25%. PREDOMINANT SEX: Female male, approximately 2:1. The highest risk for suicide is in older Caucasian men. PREDOMINANT AGE: Studies suggest that depressive symptoms peak in midlife; however, older patients may underreport their symptoms. PEAK INCIDENCE: For people over age 65, the highest rates are in the oldest old and in medical settings. GENETICS: First-degree relatives may have increased risk. The apolipoprotein E4 allele may be associated with the onset of depressive symptoms in late life.

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Depressive Disorder, Major TREATMENT NONPHARMACOLOGIC THERAPY • In the community, the first step in treating depression is to help the family and patient recognize and understand the problem. • Interpersonal therapy (IPT) and cognitive behavioral therapy (CBT) may be useful for elderly outpatients with depression. • Electroconvulsive therapy (ECT) is effective and should be considered in cases of severe depression. • Exercise or bright light treatment can be considered for mild depressive symptoms, depression with a seasonal pattern, or as augmentation of psychotherapy or pharmacologic treatment.

PHARMACOLOGIC THERAPY • Several algorithms for the treatment of depression have been studied and psychiatric practice guidelines have been published. • Many depressed geriatric patients prefer to receive treatment in the primary care setting, which has led to investigations toward improving this care. • Treatment algorithms often begin with the use of an antidepressant medication. • When choosing a medication, first consider comorbid medical conditions. Avoid agents contraindicated for specific conditions (e.g., do not use bupropion for patients with a history of seizure). • Selective serotonin reuptake inhibitors (SSRIs) are generally used first line for geriatric patients. Some of the SSRIs have fewer cytochrome P450 hepatic enzyme system interactions (citalopram, escitalopram, and sertraline). Use caution and watch for SSRI-induced hyponatremia. • Tricyclic antidepressants (TCAs) are used less frequently in the elderly due to potential anticholinergic and cardiac effects. • A medication’s side effect profile can occasionally be of benefit. For example, mirtazapine can be sedating and may also increase appetite, making it a logical choice for depressive symptoms associated with prominent anorexia and insomnia. • Monoamine oxidase inhibitors (MAOIs) are used only in severely treatment-refractory cases. • Lithium can be considered for the treatment of an MDE if the patient has a history of bipolar disorder; however use caution due to the potential for drug-induced delirium. Use low doses and target lower serum concentrations for older patients.

• In the outpatient setting, start antidepressants at half the general adult starting dose. Reassess regularly and titrate the dosage for maximum benefit. • If side effects persist and prevent use of a medication beyond the lowest starting dose, switch to another agent. Otherwise, attempt to complete an adequate trial. For example, reassess depressive symptoms, medical comorbidity, drug dose, and side effects every 2 to 4 weeks during acute outpatient treatment and gradually increase the medication to the highest tolerated dose, up to 50% of the maximum recommended dose, and maintain this for 4 to 6 weeks. If inadequate response persists, refer to psychiatry.

COMBINED TREATMENTS • For geriatric depression, combined treatment with medication and IPT may be more effective than either treatment alone. • Combined treatment is preferred for moderate to severe nonpsychotic depression. For mild cases of depression, either medication or psychotherapy alone may be adequate. • For psychotic depression, patients should be treated by a psychiatrist in an inpatient setting. Treatment with both antidepressant and antipsychotic medication is recommended. ECT is an alternative.

COMPLEMENTARY & ALTERNATIVE TREATMENT • St. John’s wort (Hypericum) is used in Europe as a pharmacologic treatment for depression. It can be purchased over-the-counter in the U.S. but is not approved by the FDA as a treatment for depression. People taking St. John’s wort should be advised of potential drug interactions with other antidepressants.

DISPOSITION • The goals of treatment are to promote remission and decrease the risk of recurrence. • Continued treatment after remission decreases the likelihood of relapse. For patients who experience their first episode of depression in late life, the general recommendation is at least 1 year symptom free on medication, then reassess. Inform patients of the increased risk for relapse if medication is discontinued. If a decision is made to discontinue treatment, then wean off slowly to prevent withdrawal symptoms and complete a risk assessment in association with each dose reduction. • The time to recurrence may be shorter in the elderly.

• Patients with a history of more than one MDE should remain on antidepressant treatment. • Patients with significant cognitive impairment should receive a comprehensive neuropsychologic assessment.

REFERRAL • Medical inpatients with undertreated depression can benefit from psychiatric consultation. • Psychologists or care managers can augment pharmacologic treatment provided by a primary care clinician. • Patients should be seen by a psychiatrist if severe symptoms are present or initial adequate treatment is not effective. • If significant suicidal thoughts are reported or suspected, an urgent or emergent psychiatric evaluation should be obtained. If necessary, most states allow facilities to hold a patient involuntarily until a thorough examination can be conducted.

PEARLS & CONSIDERATIONS CAUTION • Any mention of suicidal thoughts should prompt immediate careful assessment. • Risk assessment:  Assess risk factors for dangerous behavior, including: 1. Current suicidal or homicidal thoughts and any specific plans 2. Psychotic symptoms or disorganized thinking 3. Personal or family history of self-injury, violence, psychiatric illness, or substance abuse 4. Comorbid medical illness 5. Hopelessness 6. Access to weapons 7. Social isolation  Conduct this assessment in the following circumstances: 1. When depression is first evaluated 2. When medications are adjusted or treatment is changed 3. At any follow-up when an acute worsening or suicidal thoughts are noted • Additional treatment recommendations and results of the NIH Consensus Development Conference on Depression in Late Life can be found in Alexopoulos (2004).

PATIENT/FAMILY EDUCATION • Community programs can help elders understand how to recognize depression. • Support groups and other resources for both patients and family caregivers

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SUGGESTED READINGS

Washington, DC, 2002, American Psychiatric Publishing. Koenig HG, Blazer DG: Mood disorders. In Blazer DG, Steffens DC, Busse EW (eds): The American Psychiatric Publishing Textbook of Geriatric Psychiatry, Washington, DC, 2004, American Psychiatric Publishing, 241-268. Mueller TI et al: The course of depression in elderly patients, Am J Geriatr Psychiatry 12:22-29, 2004. AUTHOR: GARY EPSTEIN-LUBOW, M.D.

DISEASES AND DISORDERS

Alexopoulos GS: Late-life mood disorders. In Sadavoy J et al. (eds): Comprehensive Textbook of Geriatric Psychiatry, New York, 2004, WW Norton, pp 609-654. American Psychiatric Association, Practice Guidelines: www.psych.org/psych_pract/ treatg/pg/prac_guide.cfm

Bruce ML et al: Reducing suicidal ideation and depressive symptoms in depressed older primary care patients: a randomized controlled trial, JAMA 291:1081-1091, 2004. Colenda CC et al: Comparing clinical practice with guideline recommendations for the treatment of depression in geriatric patients: findings from the APA practice research network, Am J Geriatr Psychiatry 11:448-457, 2003. Jacobson SA, Pies RW, Greenblatt DJ: Handbook of Geriatric Psychopharmacology,

Section I

may improve the clinical outcome of depression.

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Diabetes Mellitus BASIC INFORMATION DEFINITION • Diabetes mellitus (DM) refers to a syndrome of hyperglycemia resulting from many different causes (see “Etiology”). It can be classified into type 1 (insulin-dependent) and type 2 (noninsulin-dependent) DM. When a type 2 diabetic person needs insulin, she becomes classified as type 2 insulin requiring. Table 1-3 provides a general comparison of the two most common types of diabetes mellitus. • The American Diabetes Association (ADA) defines DM as (1) a fasting plasma glucose 126 mg/dl or (2) a nonfasting plasma glucose 200 mg/dl or (3) an oral glucose tolerance test (OGTT) 200 mg/dl in the 2-hr sample. Furthermore, the ADA also defines a value of 110 mg/dl on fasting blood sugar as the upper limit of normal for glucose. A fasting glucose between 110 mg/dl and 126 mg/dl is classified as “impaired fasting glucose” (IFG).

SYNONYMS IDDM (insulin-dependent diabetes mellitus) NIDDM (non-insulin-dependent diabetes mellitus) Type 1 diabetes mellitus (insulindependent diabetes mellitus) Type 2 diabetes mellitus (non-insulindependent diabetes mellitus)

TABLE 1-3

ICD-9CM CODES 250.0 Diabetes mellitus (NIDDM) 250.1 Insulin-dependent diabetes mellitus without complication (IDDM)

EPIDEMIOLOGY & DEMOGRAPHICS • DM affects 5% to 7% of the U.S. population. Prevalence in Pima Indians is 35%. • The vast majority of DM in geriatric patients is type 2. • Incidence increases with age, with 2% in persons ages 20 to 44 yr to 18% in persons 65 to 74 yr of age. • Diagnosis is delayed in many patients with type 2 DM. • Diabetes accounts for 8% of all blindness and is the leading cause of endstage renal disease in the U.S. • Patients with diabetes are twice as likely as nondiabetic patients to develop cardiovascular disease.

PHYSICAL FINDINGS & CLINICAL PRESENTATION 1. Symptoms of hyperglycemia: polyuria, thirst, blurred vision. 2. Physical examination varies with the presence of complications and may be normal in early stages. 3. Diabetic retinopathy: a. Nonproliferative (background diabetic retinopathy): (1) Initially: microaneurysms, capillary dilation, waxy or

hard exudates, dot and flame hemorrhages, AV shunts (2) Advanced stage: microinfarcts with cotton wool exudates, macular edema b. Proliferative retinopathy: characterized by formation of new vessels, vitreal hemorrhages, fibrous scarring, and retinal detachment 4. Cataracts and glaucoma occur with increased frequency in diabetics. 5. Peripheral neuropathy: patients often complain of paresthesias of extremities (feet more than hands); the symptoms are symmetric, bilateral, and associated with intense burning pain (particularly during the night). a. Mononeuropathies involving cranial nerves III, IV, and VI; intercostal nerves; and femoral nerves are also common. b. Physical examination may reveal: (1) Decreased pinprick sensation, sensation to light touch, and pain sensation. (2) Decreased vibration sense. (3) Loss of proprioception (leading to ataxia). (4) Motor disturbances (decreased DTR, weakness and atrophy of interossei muscles); when the hands are affected, the patient has trouble picking up small objects, dressing, and turning pages in a book. (5) Diplopia, abnormalities of visual fields.

General Comparison of the Two Most Common Types of Diabetes Mellitus

Previous terminology Age of onset Genetic predisposition

Type 1

Type 2

Insulin-dependent diabetes mellitus (IDDM), type I, juvenileonset diabetes Usually 30 yr, particularly childhood and adolescence, but any age Moderate; environmental factors required for expression; 35%-50% concordance in monozygotic twins; several candidate genes proposed

Non–insulin-dependent diabetes mellitus, type II, adult-onset diabetes Usually 40 yr, but any age

Human leukocyte antigen associations Other associations

Linkage to DQA and DQB, influenced by DRB (3 and 4) [DR2 protective] Autoimmune; Graves’ disease, Hashimoto’s thyroiditis, vitiligo, Addison’s disease, pernicious anemia

Precipitating and risk factors Findings at diagnosis

Largely unknown; microbial, chemical, dietary, other

Endogenous insulin levels

Low or absent

Insulin resistance Prolonged fast Stress, withdrawal of insulin

Only with hyperglycemia Hyperglycemia, ketoacidosis Ketoacidosis

85%-90% of patients have one and usually more autoantibodies to ICA512/IA-2/IA-2, GAD65, insulin (IAA)

Strong; 60%-90% concordance in monozygotic twins; many candidate genes proposed; some genes identified in maturity-onset diabetes of the young None known Heterogenous group, ongoing subclassification based on identification of specific pathogenic processes and genetic defects Age, obesity (central), sedentary lifestyle, previous gestational diabetes Possibly complications (microvascular and macrovascular) caused by significant preceeding asymptomatic period Usually present (relative deficiency), early hyperinsulinemia Mostly present Euglycemia Nonketotic hyperglycemia, occasionally ketoacidosis

From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders. GAD, Glutamic acid decarboxylase; IA-2/IA-2, tyrosine phosphatases; IAA, insulin autoantibodies; ICA, islet cell antibody; ICA512, islet cell autoantigen 512 (fragment of IA-2).

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IDIOPATHIC DIABETES Type 1 DM • Hereditary factors: 1. Islet cell antibodies (found in 90% of patients within the first year of diagnosis) 2. Higher incidence of HLA types DR3, DR4 3. 50% concordance in identical twins • Environmental factors: viral infection (possibly coxsackie virus, mumps virus) Type 2 DM • Hereditary factors: 90% concordance in identical twins • Environmental factor: obesity DIABETES SECONDARY TO OTHER FACTORS • Hormonal excess: Cushing’s syndrome, acromegaly, glucagonoma, pheochromocytoma • Drugs: glucocorticoids, diuretics • Insulin receptor unavailability (with or without circulating antibodies) • Pancreatic disease: pancreatitis, pancreatectomy, hemochromatosis • Genetic syndromes: hyperlipidemias, myotonic dystrophy, lipoatrophy • Gestational diabetes • Polycystic ovary syndrome

Diagnosis is made on the basis of the following tests and should be confirmed by repeated testing on a different day: 1. Fasting glucose 126 mg/dl (ADA criteria) 2. Nonfasting plasma glucose 200 mg/dl • Use of glycosylated hemoglobin (Hb A1c) level is not recommended for diagnosis at this time by the ADA because of lack of standardization of hemoglobin A1c values and the imperfect correlation between HbA1c and fasting plasma glucose levels. However, some physicians use this test to make the diagnosis of diabetes mellitus if the random plasma glucose is 200 mg/dl and the hemoglobin A1c level is 2 standard deviations above the laboratory mean. • Creatinine, urine microalbumin, and lipids should be monitored yearly. • TSH should be checked from time to time (perhaps less than yearly).

DIFFERENTIAL DIAGNOSIS • Diabetes insipidus • Stress hyperglycemia • Diabetes secondary to hormonal excess, drugs, pancreatic disease

TREATMENT NONPHARMACOLOGIC THERAPY 1. Diet a. Calories (1) The diabetic patient can be started on 15 calories/lb of ideal body weight; this number can be increased to 20 calories/lb for an active person and 25 calories/lb if the patient does heavy physical labor. (2) The calories should be distributed as 55% to 60% carbohydrates, 25% to 35% fat, and 15% to 20% protein. (3) The emphasis should be on complex carbohydrates rather than simple and refined starches and on polyunsaturated instead of saturated fats in a ratio of 2:1. b. Seven food groups (1) The exchange diet of the ADA includes protein, bread, fruit, milk, and low- and intermediate-carbohydrate vegetables. (2) The name of each exchange is meant to be all inclusive (e.g., cereal, muffins, spaghetti, potatoes, rice are in the bread group; meats, fish, eggs, cheese, peanut butter are in the protein group).

(3) The glycemic index compares the rise in blood sugar after the ingestion of simple sugars and complex carbohydrates with the rise that occurs after the absorption of glucose; equal amounts of starches do not give the same rise in plasma glucose (pasta equal in calories to a baked potato causes less of a rise than the potato): thus, it is helpful to know the glycemic index of a particular food product. (4) Fiber: insoluble fiber (bran, celery) and soluble globular fiber (pectin in fruit) delay glucose absorption and attenuate the postprandial serum glucose peak; they also appear to lower the elevated triglyceride level often present in uncontrolled diabetics. 2. Exercise increases the cellular glucose uptake by increasing the number of cell receptors. The following points must be considered: a. Exercise program must be individualized and built up slowly. b. Insulin is more rapidly absorbed when injected into a limb that is then exercised, and this can result in hypoglycemia. 3. Weight loss: to ideal body weight if the patient is overweight. This is the single most important goal of the nonpharmacologic arm of management.

PHARMACOLOGIC THERAPY • When the previous measures fail to normalize the serum glucose, oral hypoglycemic agents (e.g., metformin, glitazones, or a sulfonylurea) should be added to the regimen in type 2 DM. Table 1-4 describes commonly used oral hypoglycemic agents. The sulfonamides and the biguanide metformin are the oldest and most commonly used classes of hypoglycemic drugs. • The goal of treatment is to achieve a stable Hb A1c under 6.5%. • Metformin’s primary mechanism is to decrease hepatic glucose output. Because metformin does not produce hypoglycemia when used as a monotherapy, it is preferred for most patients. It is contraindicated in patients with renal insufficiency and used with caution in patients with chronic liver disease or CHF. • Pioglitazone and rosiglitazone increase insulin sensitivity and are useful as single agents or in addition to other agents in type 2 diabetics whose hyperglycemia is inadequately controlled. Serum transaminase levels should be obtained before starting therapy and monitored periodically. Edema is a common complication.

DISEASES AND DISORDERS

ETIOLOGY

DIAGNOSIS

Section I

6. Autonomic neuropathy: a. GI disturbances: esophageal motility abnormalities, gastroparesis, diarrhea (usually nocturnal). b. GU disturbances: neurogenic bladder (hesitancy, weak stream, and dribbling), impotence. c. Orthostatic hypotension: postural syncope, dizziness, light-headedness 7. Nephropathy: pedal edema, pallor, weakness, uremic appearance. Neuropathy can be detected with a simple exam of the lower extremities using a 10-g monofilament to test sensation. 8. Foot ulcers: occur frequently and are usually secondary to peripheral vascular insufficiency, repeated trauma (unrecognized because of sensory loss), and superimposed infections. If a diabetic foot ulcer has been present for weeks and foot pulses are palpable, neuropathy should be considered a major cause. 9. Neuropathic arthropathy (Charcot’s joints): bone or joint deformities from repeated trauma (secondary to peripheral neuropathy). 10. Necrobiosis lipoidica diabeticorum: plaquelike reddened areas with a central area that fades to white-yellow found on the anterior surfaces of the legs; in these areas the skin becomes very thin and can ulcerate readily. 11. May be diagnosed as a result of screening or incidentally.

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Diabetes Mellitus TABLE 1-4

Oral Antidiabetic Agents as Monotherapy

Generic name

Mode of action

Sulfonylureas

Biguanides

-Glucosidase inhibitors

Glimepiride, glyburide, glipizide, chlorpropamide, tolbutamide ↑ ↑ Pancreatic insulin secretion chronically

Metformin

Preferred patient type

Diagnosis age 30 yr, lean, diabetes 5 yr, insulinopenic

Therapeutic effects ↓ HBA1c* (%) ↓ FPG* (mg/dl) ↓ PPG* (mg/dl) Insulin levels Weight Lipids

1-2 50-70 ~90 ↑ ↑ —

Thiazolidinediones

Meglitinides

Acarbose, miglitol

Rosiglitazone, pioglitazone

Repaglinide, nateglinide

↓ ↓ HGP; ↓ peripheral IR; ↓ intestinal glucose absorption Overweight, IR, fasting hyperglycemia, dyslipidemia

Delays PP digestion of carbohydrates and absorption of glucose PP hyperglycemia

↓ ↓ Peripheral IR; ↑ ↑ glucose disposal; ↓ HGP

↑ ↑ Pancreatic insulin secretion acutely

Overweight, IR, dyslipidemia, renal dysfunction

PP hyperglycemia, insulinopenic

1-2 50-80 80 — –/↓ ↓ LDL ↓ ↓ TG

0.5-1 15-30 40-50 — —

0.8-1 25-50 — — –/↑ ↑ Large “fluffy” LDL ↓ ↓ TG ↑ HDL Edema

1-2 40-80 30 ↑ ↑

— Hypoglycemia (lowrisk)

Side effects

Hypoglycemia

Diarrhea, lactic acidosis

Abdominal pain, flatulence, diarrhea

Dose(s)/day Maximum daily dose (mg)

1-3 Depends on agent

2-3 2550

1-3 150 (60-kg bw) 300 ( 60-kg bw)

1

1-4

Depends on agent

Range/dose (mg)

Depends on agent

500-1000

Depends on agent

Optimal administration time

~30 min premeal (some with food, others on empty stomach) Hepatic/renal, fecal

With meal

25-50 (60-kg bw) 25-100 ( 60-kg bw) With first bite of meal

16 (repaglinide), 360 (nateglinide) 0.5-4 (repaglinide), 60, 120 (nateglinide) Preferably 15 (0-30 min) premeals (omit if no meal)

Only 2% absorbed/fecal

Hepatic/fecal

Main site of metabolism/ excretion

Not metabolized/ renal

With meal (breakfast)

Hepatic/fecal

Modified from Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders. ↑, Increased; ↓, decreased; —, unchanged; bw, body weight; FPG, fasting plasma glucose; HDL, high-density lipoprotein; HGP, hepatic glucose production; IR, insulin resistance; LDL, low-density lipoprotein; PP, postprandial; PPG, postprandial plasma glucose; TG, triglyceride. *Values combined from numerous studies; values are also dose dependent.

• Sulfonylureas and meglitinides are insulin secretagogues. The latter work best when given before meals because they increase the postprandial output of insulin from the pancreas. All sulfonylureas are relatively contraindicated in patients allergic to sulfa. • Acarbose and miglitol work by competitively inhibiting pancreatic amylase and small intestinal glucosidases delay gastrointestinal absorption of carbohydrates, thereby reducing alimentary hyperglycemia. The major side effects are flatulence, diarrhea, and abdominal cramps. • Insulin is indicated for the treatment of all type 1 and type 2 DM patients whose diabetes cannot be adequately controlled with diet and oral agents. Table 1-5 describes commonly used

types of insulin. The risks of insulin therapy include weight gain, hypoglycemia, and, in rare cases, allergic or cutaneous reactions. Replacement insulin therapy should mimic normal release patterns. Approximately 50% to 60% of daily insulin should be a basal type consisting of a long-acting insulin (NPH, Ultralente, glargine) injected once or twice daily; the remaining 40% to 50% should be short-acting or rapidacting insulin (regular, aspart, lispro) to cover mealtime carbohydrates and correct elevated current glucose levels. Among long-acting insulins, oncedaily bedtime insulin glargine is as effective as once- or twice-daily NPH but has a lower risk of nocturnal hypoglycemia and less weight gain. When using short-acting insulins, in-

•

•

•

•

sulin aspart and insulin lispro are more effective in lowering postprandial glucose levels than regular insulin. Combination therapy of various hypoglycemic agents is commonly used when monotherapy results in inadequate glycemic control. Continuous subcutaneous insulin infusion (CSII, or insulin pump) provides better glycemic control than does conventional therapy and comparable to or slightly better control than multiple daily injections. Low-dose ASA to decrease the risk of cerebrovascular disease is beneficial for diabetics over age 30 with other risk factors (hypertension, dyslipidemia, smoking, obesity). A fasting serum lipid panel should be obtained yearly on all adult diabetic

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Types of Insulin

Lispro†

Short acting

Regular Lente NPH Ultralente 70/30 50/50

Intermediate acting Long acting‡ Mixed, short/intermediate acting

Onset* (hr)

Peak* (hr)

Duration* (hr)

Blood glucose (BG) nadir* (hr)

0-0.2

0.2-0.5

0.5-2

5 4-8 (16)

2-4 3-7 (Pre-next meal)

16-26 24-36

6-13 10-28

16-24

3-12

0.5-(1) 0.5-(1)

0.3-1 1-2 1-3 4-6

2-6 4-12 6-15 8-30

0.5-(1)

0.5-1

3-12

0.5-(1)

From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders. 70/30, 70% NPH, 30% regular; 50/50, 50% NPH, 50% regular; NPH, neutral protamine Hagedorn. *Times depend on several factors including dose, anatomic site of injection, method (SQ, IM, IV), duration of diabetes, degree of insulin resistance, level of activity, and body temperature. Some time ranges are wide to include data from several separate studies. Preprandial injection depends on premeal BG values as well as insulin type. If BG is low, may need to inject insulin and eat immediately (carbohydrate portion of meal first). If BG is high, may delay meal after insulin injection and eat carbohydrate portion last. †Insulin analogue with reversal of lysine and proline at positions 28 and 29 on the  chain. ‡Insulin glargine [rDNA origin] is a newer, once-daily insulin analog (Lantus) that provides 24-hour basal glucose-lowering with once-a-day bedtime dosing. Onset of action is 2-3 hr, duration of action is 24 hr.

patients. Strict lipid control (LDL 70 mg/dl) is indicated in all diabetics. Use of statins is often necessary to achieve therapeutic goals.

DISPOSITION The Diabetes Control and Complications Trial (DCCT) proved that intensive treatment decreases the development and progression of complications of DM. In this trial, the risks of retinopathy, nephropathy, and neuropathy were decreased by 35% to 90%. Each patient should be made aware of these findings. The United Kingdom Prospective Diabetes Study (UKPDS) confirmed the benefit of tight glycemic control, but also demonstrated that blood pressure control in patients with DM is as important as glycemic control. Target BP control in patients with DM is 130/80 or lower. • Retinopathy occurs in approximately 15% of diabetic patients after 15 yr and increases 1%/yr after diagnosis. • The frequency of neuropathy in type 2 diabetics approaches 70% to 80%. Gabapentin (900-3600 mg/day) is effective for the symptomatic treatment of peripheral neuropathic pain. Amitriptyline, duloxetine, and carbamazepine can also be effective. • Nephropathy occurs in 35% to 45% of patients with type 1 DM and in 20% of type 2 DM. The first sign of renal involvement in patients with DM is most often microalbuminuria, which is classified as incipient nephropathy. ACE inhibitors are effective in slowing the progression of renal disease in both type 1 and type 2 DM, independently of their reduction in blood pressure. ARBs and nondihydropyridine calcium channel blockers are also effective in protecting against the progression of

nephropathy in diabetics, especially in type 2 DM. • Infections are generally more common in diabetics because of multiple factors, such as impaired leukocyte function, decreased tissue perfusion secondary to vascular disease, repeated trauma because of loss of sensation, and urinary retention secondary to neuropathy. • Hyperosmolar coma is described in detail in Section I.

REFERRAL • Diabetic patients should be advised to have annual ophthalmologic examination. In type 1 DM, ophthalmologic visits should begin within 3 to 5 years, whereas type 2 DM patients should be seen from disease onset. • Podiatric care can significantly reduce the rate of foot infections and amputations in patients with DM. Noninfected neuropathic foot ulcers require debridement and reduction of pressure.

PEARLS & CONSIDERATIONS COMMENTS • Because normalization of serum glucose level is the ultimate goal, all patients should measure their blood glucose unless contraindicated by senility or blindness. • For blood glucose monitoring, glucose oxidase strips are used in conjunction with a meter to give a digital reading. The testing can be done once-a-day, but the time should be varied each day so that over a period of time the serum glucose level before meals and at bedtime can be assessed frequently without pricking the patient’s fingers four times daily.

• Glycosylated hemoglobin should be measured at least twice yearly; measurement of microalbumin in the urine on a yearly basis is also recommended.

SUGGESTED READINGS American Diabetes Association: Position statement: standards of medical care for patients with diabetes mellitus, Diabetes Care 25:S33, 2002. Barr RG et al: Tests of glycemia for the diagnosis of type 2 diabetes mellitus, Ann Intern Med 137:263, 2002. Beckman JA et al: Diabetes and atherosclerosis, JAMA 287:2570, 2002. Boulton AJM et al: Neuropathic diabetic foot ulcers, N Engl J Med 351:48-55, 2004. DeWitt DE, Hirsch IB: Outpatient insulin therapy in type 1 and type 2 DM, JAMA 289:2254, 2003. Holmboe ES: Oral antihyperglycemic therapy for type 2 diabetes, JAMA 287:373, 2002. Mayfield J, White R: Insulin therapy for type 2 DM: rescue, augmentation, and replacement of beta cell function, Am Fam Physician 70:489-512, 2004. Nathan DM: Initial management of glycemia in type 2 diabetes mellitus, N Engl J Med 347:1342, 2002. Remuzzi G et al: Nephropathy in patients with type 2 diabetes, N Engl J Med 346:1145, 2002. Stern MP et al: Identification of persons at high risk for type 2 diabetes mellitus: do we need the oral glucose tolerance test? Ann Intern Med 136:575, 2002. U.S. Preventive Services Task Force: Screening for type 2 DM in adults: recommendations and rationale, Ann Intern Med 138:212, 2003. Zandbergen AM et al: Effect of losartan on microalbuminuria in normotensive patients with type 2 DM, Ann Intern Med 139:90, 2003. AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

Rapid acting

Preprandial injection timing* (hr)

Section I

Insulin type

Generic name

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Diverticular Disease (Diverticulosis, Diverticulitis)

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BASIC INFORMATION DEFINITION • Colonic diverticula are herniations of mucosa and submucosa through the muscularis. They are generally found along the colon’s mesenteric border at the site where the vasa recta penetrates the muscle wall (anatomic weak point). • Diverticulosis is the asymptomatic presence of multiple colonic diverticula. • Diverticulitis is an inflammatory process or localized perforation of diverticulum. ICD-9CM CODES 562.10 Diverticulosis of colon 562.11 Diverticulitis of colon

EPIDEMIOLOGY & DEMOGRAPHICS • Incidence of diverticulosis in the general population is 35% to 50%. • Diverticulosis is more common in Western countries, affecting 30% of people 40 yr and 50% of people 70 yr.

PHYSICAL FINDINGS & CLINICAL PRESENTATION

vated in acute bleeding secondary to reticulocytosis.

IMAGING STUDIES • Barium enema will demonstrate multiple diverticula and muscle spasm (“sawtooth” appearance of the lumen) in patients with painful diverticular disease. Barium enema can be hazardous and should not be performed in the acute stage of diverticulitis because it may produce free perforation. • A CT scan of the abdomen can be used to diagnose acute diverticulitis; typical findings are thickening of the bowel wall, fistulas, or abscess formation. • Evaluation of suspected diverticular bleeding: 1. Arteriography if the bleeding is faster than 1 ml/min (advantage: the possible infusion of vasopressin directly into the arteries supplying the bleeding, as well as selective arterial embolization; disadvantages: its cost and invasive nature) 2. Technetium-99m sulfa colloid 3. Technetium-99m labeled RBC (can detect bleeding rates as low as 0.12 to 5 ml/min)

• Physical examination in patients with diverticulosis is generally normal. • Painful diverticular disease can present with LLQ pain, often relieved by defecation; location of pain may be anywhere in the lower abdomen because of the redundancy of the sigmoid colon. • Diverticulitis can cause muscle spasm, guarding, and rebound tenderness predominantly affecting the LLQ.

TREATMENT

ETIOLOGY

TREATMENT OF DIVERTICULITIS: • Mild case: broad-spectrum PO antibiotics (e.g., ciprofloxacin 500 mg bid to cover aerobic component of colonic flora and metronidazole 500 mg q6h for anaerobes) and liquid diet for 7 to 10 days • Severe case: NPO and IV antibiotic therapy a. Ampicillin-sulbactam (Unasyn) 3 g IV q6h or b. Piperacillin-tazobactam (Zosyn) 4.5 g IV q8h or c. Ciprofloxacin 400 mg IV q12h plus metronidazole 500 mg IV q6h or d. Cefoxitin 2 g IV q8h plus metronidazole 500 mg IV q6h • Life-threatening case: Imipenem 500 mg IV q6h or meropenem 1 g IV q8h • Surgical treatment consisting of resection of involved areas and reanastomosis (if feasible); otherwise a diverting colostomy with reanastomosis

• Diverticular disease is believed to be secondary to low intake of dietary fiber.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • •

Irritable bowel syndrome IBD Carcinoma of colon Endometriosis Ischemic colitis Infections (pseudomembranous colitis, appendicitis, pyelonephritis, PID) • Lactose intolerance

LABORATORY TESTS • WBC count in diverticulitis reveals leukocytosis with left shift. • Microcytic anemia can be present in patients with chronic bleeding from diverticular disease. MCV may be ele-

NONPHARMACOLOGIC THERAPY • Increase in dietary fiber intake and regular exercise to improve bowel function • NPO and IV hydration in severe diverticulitis; NG suction if ileus or small bowel obstruction is present

ACUTE GENERAL Rx

performed when infection has been controlled; surgery should be considered in patients with: 1. Repeated episodes of diverticulitis (two or more) 2. Poor response to appropriate medical therapy (failure of conservative management) 3. Abscess or fistula formation 4. Obstruction 5. Peritonitis 6. Immunocompromised patients, first episode in young patient (40 yr old) 7. Inability to exclude carcinoma (10% to 20% of patients diagnosed with diverticulosis on clinical grounds are subsequently found to have carcinoma of the colon) DIVERTICULAR HEMORRHAGE: 70% of diverticular bleeding occurs in the right colon. 1. Bleeding is painless and stops spontaneously in the majority of patients (60%); it is usually caused by erosion of a blood vessel by a fecalith present within the diverticular sac. 2. Medical therapy consists of blood replacement and correction of volume and any clotting abnormalities. 3. Colonoscopic treatment with epinephrine injections, bipolar coagulation, or both may prevent recurrent bleeding and decrease the need for surgery. 4. Surgical resection is necessary if bleeding does not stop spontaneously after administration of 4 to 5 U of PRBCs or recurs with severity within a few days; if attempts at localization are unsuccessful, total abdominal colectomy with ileoproctostomy may be indicated (high incidence of rebleeding if segmental resection is performed without adequate localization).

CHRONIC Rx Asymptomatic patients with diverticulosis can be treated with a high-fiber diet or fiber supplements.

DISPOSITION • Most patients with diverticulitis respond well to antibiotic management and bowel rest. Up to 30% of patients with diverticulitis will eventually require surgical management. • Diverticular bleeding can recur in 15% to 20% of patients within 5 yr.

REFERRAL Surgical referral when considering resection (see Acute General Rx) AUTHOR: FRED F. FERRI, M.D.

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Dupuytren’s Contracture BASIC INFORMATION

ICD-9CM CODES 728.6 Dupuytren’s contracture

ETIOLOGY Unknown

EPIDEMIOLOGY & DEMOGRAPHICS

DIAGNOSIS

PREVALENCE: Varies depending on nationality (see Pearls & Considerations) PREVALENT AGE: Fifth decade and up PREVALENT SEX: Male:female ratio of 10:1

DIFFERENTIAL DIAGNOSIS

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Usually asymptomatic • Most common complaints: deformity and interference with the use of the hand by the flexed, contracted fingers (Fig. 1-14) • Process usually begins in the ulnar side of the hand, often starting at the ring finger • Isolated painless nodules that eventually harden and mature into a longitudinal cord that extends into the finger

• Soft tissue tumor, tendon cyst • Trigger finger

TREATMENT NONPHARMACOLOGIC THERAPY • Stretching exercises • Local heat

DISPOSITION Rate of development is variable.

REFERRAL • If joint contracture begins to develop • For excision of rare nodule that is painful (at any stage)

COMMENTS • Dupuytren’s contracture develops earlier and more often in certain families. • The disorder is more common in Scandinavians, and some Northern Europeans have a 25% prevalence over age 60 yr. • About 5% of patients develop a similar condition elsewhere, such as Peyronie’s disease or Ledderhose disease (involvement of the plantar fascia). • Soft tissue “pads” in the knuckles may also be present. • Individuals with these additional findings are considered to have Dupuytren’s diathesis, and their disease is generally more severe and recurrent.

SUGGESTED READINGS Frank PL: An update on Dupuytren’s contracture, Hosp Med 62:678, 2001. Khan AA et al: The role of manual occupation in the aetiology of Dupuytren’s disease in men in England and Wales, J Hand Surg 299(1):12, 2004. McFarlane RM: On the origin and spread of Dupuytren’s disease, J Hand Surg 27:385, 2002. Ragsowansi RH, Britto JA: Genetic and epigenetic influence on the pathogenesis of Dupuytren’s disease, J Hand Surg 26:1157, 2001. Thurston AJ: Dupuytren’s disease, J Bone Joint Surg Br 85(4):469, 2003. AUTHOR: LONNIE R. MERCIER, M.D.

FIGURE 1-14 Dupuytren’s contracture. A flexion deformity of the finger is present, with nodular thickening of the fascia to the ring finger.

DISEASES AND DISORDERS

Dupuytren’s contracture is a disease of the palmar fascia characterized by nodular fibroblastic proliferation that often results in progressive contractures of the fascia and flexion deformity of the fingers.

PEARLS & CONSIDERATIONS

Section I

DEFINITION

• Lesion often begins in the distal palmar crease • Overlying skin adherent to the fascia • Later stages: fibrous cord begins to contract and pull the finger into flexion • Possible involvement of other fingers, particularly small finger

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Dysphagia BASIC INFORMATION DEFINITION Dysphagia is defined as difficulty in swallowing either from problems in transferring the food bolus from the oropharynx to the upper esophagus or impairment in transport of the bolus through the body of the esophagus.

SYNONYMS Oropharyngeal dysphagia Esophageal dysphagia ICD-9CM CODES 787.2 Dysphagia 438.82 Dysphagia, late effects CVA 530.3 Esophageal stricture/stenosis 787.2 Dysphagia 530.0 Achalasia

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE • 7% to 10% people over age 50. • 30% to 40% of nursing home patients experience some amount of dysphagia. • 25% of hospitalized patients experience problems swallowing.

PHYSICAL FINDINGS & CLINICAL PRESENTATION HISTORY: A thorough history often gives clues to the site of the lesion and nature of the problem: • Are symptoms progressive or intermittent in nature? • What is the duration and course of the dysphagia? • What type of food causes dysphagia? • Describe the symptom; patients often report food “sticking” after swallowing or have to repeat swallows to relieve the dysphagia. • Is there any substernal pain or heartburn on swallowing? • Odynophagia—painful swallowing associated with sharp substernal pain • Medication history. • Are there any associated symptoms (e.g., aspiration, weight loss, nasal regurgitation, unilateral wheezing, chest pain)? PHYSICAL EXAMINATION • Skin for signs of collagen vascular disease such as scleroderma • Oral cavity and pharynx for lesions causing obstruction or painful swallowing (mucositis) • Neck and axilla for thyroid swelling, lymph nodes, or tumor • Pulmonary for unilateral wheezing, aspiration pneumonia, foreign body, mass • Neurologic for signs of a neuromuscular disease, cerebrovascular accident, Parkinson’s disease, or tardive dyskinesia • Abdomen for liver and other organomegaly or masses

ETIOLOGY • Oropharyngeal dysphasia: may be due to a variety of mechanical and neuromuscular conditions. • Esophageal dysphagia: may be due to mechanical lesions or motility disorders.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Causes of oropharyngeal dysphagia:  Neurologic disorders—ALS, dementia, MS, Parkinson’s disease, cerebrovascular accident, tardive dyskinesia, Huntington’s disease  Muscular and rheumatologic disorders—myopathies, polymyositis, Sjögren’s syndrome  Metabolic disorders—thyrotoxicosis, Cushing’s, amyloidosis  Medication side effects—anticholinergics, metoclopramide, amiodarone, HMG COA reductase inhibitors, phenothiazines, over-the-counter medications, bisphosphonates  Infectious disease—mucositis (Candida, herpes)  Structural disorders—Zenker’s diverticulum, esophageal tumors, postsurgical or radiation changes, cervical osteoarthritis and osteophytic impingement, pill-induced injury • Causes of esophageal dysphagia:  Mechanical lesions—intrinsic narrowing, a very large food bolus, or compression from an extrinsic source 1. Malignant stricture 2. Peptic stricture 3. Schatzki’s ring  Motility disorders—dysphagia equally for both solids and liquids, episodic, usually nonprogressive 1. Achalasia 2. Diffuse esophageal spasm 3. Scleroderma • Other causes:  Globus hystericus—a sensation of a “ball” in the throat, often underlying psychiatric causes. No abnormality in swallowing exists.

WORKUP • Swallowing evaluation by speech therapist, particularly when there is a risk of aspiration (e.g., in the context of aspiration pneumonia)

LABORATORY TESTS • Necessary in some cases of metabolic disorders (thyrotoxicosis, Cushing’s) • To identify infections (candida, herpes)

IMAGING STUDIES • Oropharyngeal dysphagia  Barium esophagography. • Esophageal dysphagia:  Upper endoscopy procedure of choice. Barium swallow an alternative.  Esophageal manometry and pH.

TREATMENT ACUTE GENERAL Rx • Oropharyngeal dysphagia:  Treat the underlying neuromuscular or metabolic disorder.  Adjust diet as tolerated.  Swallowing techniques designed to strengthen muscles used in swallowing.  Eliminate medications known to cause or worsen dysphagia (NSAIDs, potassium tablets, doxycycline, bisphosphonates).  Gastrostomy tubes—unproven if this reduces the risk of aspiration pneumonia. • Esophageal dysphagia:  Radical surgery  Motility disorders can be treated with balloon dilators for the lower esophageal sphincters or injections of botulinum toxin.  Dilation of benign strictures or rings. • Esophageal cancer:  Symptomatic relief with stents or dilation.  Ablation of tumors with laser or cautery. • Diffuse esophageal spasm:  Supportive care  Empirical trial of smooth muscle relaxants (i.e., isosorbide dinitrate, nifedipine)  Empirical trial of an antidepressant (i.e., trazodone or amitriptyline)

REFERRAL • Gastroenterology

PEARLS & CONSIDERATIONS COMMENTS • Dysphagia, heartburn, and odynophagia symptoms always indicate a primary esophageal disorder. • Persistent symptoms should be investigated by an upper endoscopy. • Consider risk of aspiration; when present, request a swallowing evaluation by a speech therapist and implement recommendations for special textured diets.

SUGGESTED READINGS American Gastroenterological Association: Medical position statement on management of oropharyngeal dysphagia, Gastroenterology 116:452, 1999. Spieker MR: Evaluating dysphagia, Am Fam Physician 61:3639, 2000. AUTHORS: SUSAN BERNER, M.D., and ARVIND MODAWAL, M.D., M.P.H., M.R.C.G.P.

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Dystonia BASIC INFORMATION

SYNONYMS Blepharospasm Oromandibular dystonia Torticollis Writer’s cramp ICD-9CM CODES 333.6 Dystonia musculorum deformans 335.7 Dystonia caused by drugs 333.7 Dystonia, torsion, symptomatic

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Estimated at 1 in 3000 persons. PREDOMINANT SEX: Cervical dystonia has a 3:2 female preponderance. PREDOMINANT AGE • Focal cervical dystonia usually has its onset in the fifth decade. • Hereditary forms may have an onset in childhood or adulthood. GENETICS: Autosomal dominant, autosomal recessive, and X-linked forms of dystonia have been identified.

DIAGNOSIS

• Heat, massage, physical therapy to relieve pain • Splints to prevent contractures

DIFFERENTIAL DIAGNOSIS • • • • •

Parkinson’s disease Progressive supranuclear palsy Wilson’s disease Huntington’s disease Drug effects

CT scan or MRI of brain if a CNS lesion is suspected

TREATMENT NONPHARMACOLOGIC THERAPY

ACUTE Rx For acute dystonic reactions to phenothiazines or butyrophenones use diphenhydramine 50 mg IV or benztropine 2 mg IV.

CHRONIC Rx

History (including family history, birth history, medication use) and physical examination

• Treatment is often ineffective. • Slowly withdraw potentially offending agents. • Diazepam, baclofen, or carbamazepine may be helpful. • Trihexyphenidyl may be helpful in tardive dyskinesia or dystonia. • Injections of botulinum toxin into the affected muscles can be used for refractory cases of focal dystonias. • Surgical procedures including myectomy, rhizotomy, or thalamotomy may be helpful for severe, refractory cases.

Focal dystonias produce abnormal sustained muscle contractions in an area of the body: • Neck (torticollis): most commonly affected site with a tendency for the head to turn to one side • Eyelids (blepharospasm): involuntary closure of the eyelids • Mouth (oromandibular dystonia): involuntary contraction of muscles of the mouth, tongue, or face • Hand (writer’s cramp) (Fig. 1-15) Generalized dystonia affects multiple areas of the body and can lead to marked joint deformities. • Exact pathophysiology is unknown; thought to involve abnormalities of basal ganglia. • Hereditary forms have been described, including the severe progressive form, dystonia musculorum deformans.

IMAGING STUDIES

WORKUP

PHYSICAL FINDINGS & CLINICAL PRESENTATION

ETIOLOGY

• Usually not helpful for establishing diagnosis • Serum ceruloplasmin if Wilson’s disease is suspected

DISPOSITION Spontaneous remission of focal cervical dystonia can occur, but dystonia is generally progressive and pharmacologic therapy is often ineffective.

REFERRAL To neurologist for severe or refractory cases

SUGGESTED READINGS Tan N-C et al: Hemifacial spasm and involuntary facial movements, QJM 95(8):493, 2002. FIGURE 1-15 Focal dystonia of the distal right arm. (From Goldman L, Bennett JC [eds]: Cecil textbook of medicine, ed 21, Philadelphia, 2000, WB Saunders.)

AUTHOR: MARK J. FAGAN, M.D.

DISEASES AND DISORDERS

Dystonia is characterized by involuntary muscle contractions (sustained or spasmodic) that lead to abnormal body movements or postures. Dystonia can be generalized or focal.

LABORATORY TESTS Section I

DEFINITION

• Sporadic or idiopathic forms occur. • Dystonia can occur secondary to other diseases such as CNS disease, hypoxia, kernicterus, Huntington’s disease, Wilson’s disease, Parkinson syndrome, lysosomal storage diseases. • Acute dystonia can occur following treatment with drugs that block dopamine receptors, such as phenothiazines or butyrophenones. • Tardive dyskinesia or dystonia can result from long-term treatment with antipsychotic drugs such as phenothiazines or butyrophenones.

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Endometrial Cancer BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Endometrial cancer is a malignant transformation of endometrial stroma or glands typified by irregular nuclear membranes, nuclear atypia, mitotic activity, loss of glandular pattern, irregular cell size (Fig. 1-16).

• • • • • •

SYNONYMS

WORKUP

Uterine cancer (some forms)

• Complete history and physical examination including pelvic exam to assess uterine size • Endometrial biopsy or dilation and curettage • Assessment of operative risk

ICD-9CM CODES 182 Malignant neoplasm of body of uterus

EPIDEMIOLOGY & DEMOGRAPHICS

Atypical hyperplasia Other genital tract malignancy Polyps Atrophic vaginitis Granuloma cell tumor Fibroid uterus

LABORATORY TESTS

INCIDENCE: 21.2 cases/100,000 persons; approximately 30,000 new cases annually PREDOMINANCE: Median age at onset: 60 yr RISK FACTORS: Obesity, diabetes, nulliparity, early menarche and late menopause, unopposed estrogen therapy, tamoxifen use, endometrial atypical hyperplasia

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Abnormal uterine bleeding or postmenopausal bleeding in 90% • Pyometra or hematometra • Abnormal Pap smear • Enlarged uterus

ETIOLOGY Endogenous or exogenous chronic unopposed estrogen stimulation of the endometrium

• CBC • Chemistry profile including liver function tests • Consider CA-125 level

IMAGING STUDIES • Chest x-ray examination • Possible CT scan, BE, or pelvic ultrasound  Pelvic ultrasound to assess endometrial thickness may complement a pelvic examination. • Endovaginal ultrasound in postmenopausal women with vaginal bleeding

• Surgery consists of pelvic washings, total abdominal hysterectomy and bilateral salpingo-oophorectomy, omental biopsy, and selective pelvic and periaortic lymphadenectomy, depending on stage and grade. • Brachytherapy or teletherapy are added in an advanced stage.

ACUTE GENERAL Rx • Chemotherapy (cisplatin, Adriamycin) or tamoxifen may also be used.

CHRONIC Rx • Physical and pelvic examination every 3 mo for 2 yr, then every 6 mo for 2 yr, annually thereafter. • Yearly Pap smear. • Hormone replacement can be considered in low-risk patients (stage I or early stage II) only to manage bothersome menopausal symptoms that fail to respond to other treatment modalities.

DISPOSITION The majority of cases present early, where the 5-yr survival is generally good: Stage I 75% to 100% Stage II 60% Stage III 50% Stage IV 20% Some histologic types (clear cell, serous papillary) have poorer survival rates.

REFERRAL

TREATMENT NONPHARMACOLOGIC THERAPY • Surgery is the mainstay of treatment, with or without radiation, depending on tumor stage and grade.

A gynecologist may manage early-stage disease; otherwise refer to a gynecologic oncologist.

PEARLS & CONSIDERATIONS • Any vaginal bleeding in a postmenopausal woman requires ruling out endometrial cancer. • Pap smear is not a proven method to screen for endometrial cancer.

SUGGESTED READINGS

B

A

FIGURE 1-16 Carcinoma of the endometrium. A, Stage I. B, Stage III, myometrial invasion. (From Sabiston D: Textbook of surgery, ed 15, Philadelphia, 1997, WB Saunders.)

Smith-Bindman R et al: Endovaginal ultrasound to exclude endometrial cancer and other endometrial abnormalities, JAMA 280:1510, 1998. Suriano KA et al: Estrogen replacement therapy in endometrial cancer patients, Obstet Gynecol 97:555, 2001. Tabor A et al: Endometrial thickness as a test for endometrial cancer in women with postmenopausal vaginal bleeding, Obstet Gynecol 99:529, 2002. AUTHORS: GIL FARKASH, M.D., with revisions by TOM J. WACHTEL, M.D.

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Erectile Dysfunction BASIC INFORMATION Erectile dysfunction (ED) is the inability to achieve or sustain a penile erection of adequate rigidity to make intercourse possible. Impotence Male erectile disorder Sexual dysfunction (a nonspecific term) ICD-9CM CODES F52.2 Male erectile disorder DSM-IV Code: 302.72 Male erectile disorder

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE (IN U.S.) • Increases with age. • 25% for men in their 60s, 80% for men in their 80s. • The Massachusetts Male Aging Study reports a 1989 prevalence as 52%, with 9.6% of respondents with complete erectile dysfunction; in 2000, the prevalence was 44%. • Likely underestimated because of social stigma. PEAK INCIDENCE: Over 70 yr old

CLINICAL PRESENTATION • Psychogenic impotence: inability to obtain erection, inability to obtain or maintain an adequate erection, or the loss of erection before completion of sexual intercourse; nocturnal penile tumescence usually normal. • Organic impotence: inability to obtain an erection or inability to obtain an adequate erection; nocturnal penile tumescence usually abnormal. • Loss of libido suggests hypogonadism.

ETIOLOGY • Psychogenic erectile dysfunction resulting from a wide range of experiential, historical, or psychological processes. • Mental health disorders, particularly depression, widower syndrome, and performance anxiety are known psychogenic contributors. • Organic impotence resulting from a wide variety of insults to neurologic, hormonal, or vascular structures. In approximately 40% of men 50 years of age, the primary cause of ED is related to atherosclerotic disease. • Medications (antihypertensives, antidepressants, antipsychotics, histamine blockers, nicotine, alcohol, and others) are commonly causative. • Endocrinopathies such as diabetes, hypogonadism, hypothyroidism or hyperthyroidism, and hyperprolactinemia.

DIFFERENTIAL DIAGNOSIS • Psychogenic dysfunction distinguished from organic. • Etiology of organic dysfunction to be determined. • Erectile dysfunction possible in the setting of another psychiatric condition.

CHRONIC Rx

• History (often including partner report) with a focus on risk factors (e.g., smoking, alcohol) • Report of nocturnal erections • Physical examination to rule out neuronal damage, direct penile damage (e.g., Peyronie’s fibrosis), or testicular atrophy

• Psychogenic impotence: relatively uncommon and characterized objectively by nocturnal and morning erections and otherwise negative test results. PDE5 inhibitors effective in patients with depression because tissues, nerves, hormones, and vasculature normal. Full psychologic evaluation recommended before starting treatment so underlying problem is addressed. • For men failing other approaches: penile prosthesis. • Testosterone therapy in elderly hypogonadal males.

LABORATORY TESTS

DISPOSITION

WORKUP

Evaluate for endocrine abnormalities with morning serum testosterone (total and free), FSH, LH, TSH, prolactin, HbA1c or fasting glucose, and lipid panel.

OTHER STUDIES IN SELECTED CASES • Nocturnal penile tumescence very specific for distinguishing psychogenic versus organic causes. • Vascular etiologies screened by the penile-brachial pressure index (measures the loss of systolic blood pressure between the arm and penis) or with Doppler studies. • Neurogenic etiologies examined by the bulbocavernosus reflex or the pudendal-evoked response. • Intracorporeal injection of prostaglandin E1 to distinguish vascular and nonvascular etiologies (erection is achieved in patients with nonvascular etiologies).

TREATMENT NONPHARMACOLOGIC THERAPY • Various psychotherapeutic approaches; success rates decrease with advancing age and duration of symptoms. • Sex therapy and couples’ therapy are used to address technical or social issues that contribute to impotence. • Vacuum devices (70% to 90% effective) work for many men, but they are difficult to use and cumbersome.

ACUTE GENERAL Rx • Phosphodiesterase type 5 inhibitors: Sildenafil (Viagra) 50 to 100 mg, or vardenafil (Levitra) 10 to 20 mg before sexual activity, or tadafil (Cialis) 5 to 20 mg qd prn (effective for 36 hours);

• When erectile dysfunction is secondary to an organic cause, it does not remit unless the organic cause is corrected; therefore, it is usually a chronic condition. • Psychogenic acquired erectile dysfunction will remit spontaneously in 15% to 30% of the cases.

REFERRAL If psychotherapy, sex therapy, or invasive organic treatment required

PEARLS & CONSIDERATIONS • Although erectile dysfunction is not part of normal aging, both libido and quality of erection diminish with age. This can lead to performance anxiety, which sometimes can be alleviated with a short-term course of a phosphodiesterase type 5 inhibitor, which can restore (“jump-start”) sexual function particularly in older men who have been sexually inactive for a period for a variety of reasons. • It is often a good idea to involve both partners in a discussion of therapies for ED. • ED and BPH with lower urinary tract symptoms are often associated. The presence of either should prompt the clinician to inquire about the other.

SUGGESTED READINGS Fazio L et al: Erectile dysfunction: management update, Can Med Assoc Jour 170:1429, 2004. Fink HA et al: Sildenafil for male erectile dysfunction, Arch Intern Med 162:1349, 2002. Miller TA: Diagnostic evaluation of erectile dysfunction, Am Fam Physician 61:95, 2000. AUTHORS: AMAR DESAI, M.D., M.P.H., with revisions by TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

SYNONYMS

DIAGNOSIS

avoid concomitant use of nitrates and -1 blockers. • Intracavernosal injections of vasodilators (e.g., papaverine, alprostadil, or prostaglandin E1 pellet). • Oral medications such as pentoxifylline and yohimbine (limited success).

Section I

DEFINITION

• Neurogenic causes including spinal cord lesions, cortical lesions, and peripheral neuropathies.

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Esophageal Tumors BASIC INFORMATION DEFINITION Esophageal tumors are defined as benign and malignant tumors arising from the esophagus. Approximately 15% of esophageal cancers arise in the cervical esophagus, 50% in the middle third of the esophagus, and 35% in the lower third. Eighty-five percent of esophageal tumors are squamous cell carcinoma (arising from squamous epithelium). Adenocarcinomas arise from columnar epithelium in the distal esophagus, which have become dysplastic secondary to chronic gastric reflux. ICD-9CM CODES 150.8 Esophageal cancer, NEC 150.9 Esophageal cancer, NOS 230.1 Carcinoma of esophagus, in situ

EPIDEMIOLOGY & DEMOGRAPHICS Carcinomas of the esophageal epithelium, both squamous cell and adenocarcinoma, are by far the most common and important tumors of the esophagus. Benign neoplasms are much less common and include leiomyoma, papilloma, and fibrovascular polyps. Prevalence of esophageal carcinoma varies widely in different parts of world, from 7.6 cases per 100,000 persons in the U.S. to 130 cases per 100,000 persons in China. It occurs frequently within the so-called Asian “esophageal cancer belt,” extending from the southern shore of the Caspian Sea to northern China, with certain high-incidence pockets in Finland, Ireland, SE Africa, and NW France. In the U.S., 13,900 new cases and 13,000 deaths occur per year, making it the seventh leading cause of death by cancer among men. Esophageal cancer is more common among blacks than whites and has a high male:female ratio of 3:1. It usually develops in the seventh and eighth decades of life and is an illness associated with lower socioeconomic status. More than 50% of patients are diagnosed with esophageal cancer at an advanced stage (unresectable or metastatic disease).

PHYSICAL FINDINGS & CLINICAL PRESENTATION Symptoms and signs: • Dysphagia: initially occurs with solid foods and gradually progresses to include semisolids and liquids; later signs usually indicate incurable disease with tumor involving more than 60% of the esophageal circumference, and occurs in 74% of patients. • Weight loss: more than many of patients present with weight loss usually of short duration. Weight loss 10% of body mass is an independent predictor of poor prognosis.

• Hoarseness: suggests recurrent laryngeal nerve involvement • Odynophagia: an unusual symptom • Cervical adenopathy: usually involving supraclavicular lymph nodes • Dry cough: suggests tracheal involvement • Aspiration pneumonia: may signal development of a fistula between the esophagus and trachea • Massive hemoptysis or hematemesis: results from the invasion of vascular structures • Advanced disease spreads to liver, lungs, and pleura • Hypercalcemia: usually associated with squamous cell carcinoma because of the secretion of a tumor peptide similar to the parathyroid hormone

ETIOLOGY Pathogenesis of esophageal cancers is felt to be due to chronic recurrent oxidative damage from any of the following etiological agents listed below, which cause inflammation, esophagitis, increased cell turnover and ultimately, initiation of the carcinogenic process. Etiologic Agents: • Excess alcohol consumption: accounts for 80% to 90% of esophageal cancer in the U.S., with whiskey being associated with a higher incidence than wine or beer • Cigarette smoking: alcohol and tobacco use combined increase the risk substantially • Other ingested carcinogens: Nitrates (converted to nitrites): South Asia, China Smoked opiates: Northern Iran Fungal toxins in pickled vegetables • Mucosal damage: Long-term exposure to extremely hot tea Lye ingestion • Radiation-induced strictures • Chronic achalasia: incidence is seven times higher • Host susceptibility secondary to precancerous lesions: Plummer-Vinson syndrome (PatersonKelly): glossitis with iron deficiency Congenital hyperkeratosis and pitting of palms and soles • Chronic GERD leading to Barrett’s esophagus and adenocarcinoma (whites are affected more than blacks) • Possible association with celiac sprue or dietary deficiencies of molybdenum, zinc, vitamin A

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Achalasia of the esophagus Scleroderma of the esophagus Diffuse esophageal spasm Esophageal rings and webs

PHYSICAL EXAMINATION • Monitor weight • Findings are often limited to cervical and supraclavicular lymph nodes • Signs of lung consolidation from aspiration pneumonia

LABORATORY TESTS Complete blood cell count, chemistries, liver enzymes

IMAGING STUDIES • Double contrast esophagogram effectively identifies large esophageal lesions (Fig. 1-17). • In contrast to benign esophageal leiomyomata, which cause esophageal narrowing with preservation of normal mucosal pattern, esophageal carcinomas cause ragged ulcerating changes in the mucosa in association with deeper infiltration. • Smaller tumors can be missed by esophagogram, therefore esophagoscopy is recommended. • Esophagoscopy is performed to visualize tumor and obtain histopathologic confirmation. In conjunction, an endoscopic ultrasonogram is often performed to determine the depth of tumor invasion. • This population is also at risk for cancers of head, neck, and lung; therefore endoscopic inspection of larynx, trachea, and bronchi should also be considered. • Endoscopic biopsies fail to recover malignant tissue one third of the time, thus cytologic examination of tumor brushings should be routinely performed. • Examination of the fundus of the stomach via retroflexion of the endoscope is also imperative. • Chest and abdominal CT scan should be performed to determine the extent of tumor spread to mediastinum, paraaortic lymph nodes, and liver.

TREATMENT ACUTE GENERAL Rx SURGICAL RESECTION: • Surgical resection of squamous cell and adenocarcinoma of the lower third of the esophagus is done in most centers if there is no widespread metastasis. • Less than 20% of patients who survive a total resection can be expected to survive after 5 yr. Usually stomach or colon is used for esophageal replacement. POSSIBLE COMPLICATIONS OF SURGERY: Anatomic fistula (usually with colon interposition, subphrenic abscesses); respiratory complications are less common as a result of advances in surgical techniques, respiratory therapy, hyperalimentation, and anesthetic support during surgery. Cardiovascular

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Esophageal Tumors CHEMOTHERAPY: • Single agent resulted in significant tumor regression in 15% to 25% of patients and combination chemotherapy including cisplatin achieved significant tumor reduction in 30% to 60% of patients. COMBINATION CHEMOTHERAPY, RADIATION Rx, AND SURGICAL Rx: • Combination therapy has not been found to be associated with improved survival, however, many centers are using preoperative chemotherapy for many patients with esophageal cancer. • Palliative procedures such as repeated endoscopic dilation, surgical placement of feeding tube, or polyvinyl prosthesis to bypass tumors have been used for surgically unresectable patients.

DISPOSITION • Surgery: 5-yr survival rate is 48% in stages I and II, 20% in advanced stages. • Radiation therapy: 5-yr survival rate is between 6% and 20%. • Chemotherapy: Simple agent response rate 15% to 38%; combination response rate 80%. • Combined modality: 18% response rate. • Patients with stage IV disease receive palliative chemotherapy with a median survival of less than 1 year.

SUGGESTED READINGS Enzinger PC, Mayer RJ: Esophageal cancer, N Engl J Med 349:2241, 2003. Shaheen N et al: Gastroesophageal reflux, Barrett esophagus, and esophageal cancer: clinical applications, JAMA 287(15):1982, 2002. FIGURE 1-17 Barium swallow demonstrating the classic findings in cancer of the distal third of the esophagus. (From Nobel J [ed]: Primary care medicine, ed 2, St Louis, 1996, Mosby.)

AUTHORS: LYNN MCNICOLL, M.D., and MADHAVI YERNENI, M.D.

DISEASES AND DISORDERS

COMPLICATIONS OF RADIATION THERAPY: • Esophageal stricture, radiation-induced pulmonary fibrosis, transverse myelitis are the most feared complications. • Radiation-induced cardiomyopathy and skin changes occur less frequently given modern techniques. • Mucositis, GI toxicity, and myelosuppression occur frequently. • Nephrotoxicity, ototoxicity, and neurotoxicity can develop with cisplatin.

Section I

complications are by far the most common including MI, CVA, and PE. RADIATION THERAPY: • Squamous cell carcinomas are more radiosensitive than adenocarcinoma, and radiation achieves good local control and is an excellent palliative modality for obstructive symptoms. Usually employed for tumors in upper third of esophagus, often for middle third tumors as well. • About 40% of tumors cannot be destroyed even after 6000 rads. • Palliative radiation therapy for bone metastasis is also effective.

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Essential Tremor BASIC INFORMATION DEFINITION A predominantly postural and action tremor that is bilateral and tends to progress slowly over the years in the absence of other neurological abnormalities. ICD-9CM CODES 333.1 Essential tremor

EPIDEMIOLOGY & DEMOGRAPHICS About 415/100,000 in persons over 40 yr. No gender or racial predominance.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients complain of tremor that is most bothersome when writing or holding something, such as a newspaper, or trying to drink from a cup. Worsens under emotional duress and drinking liquids • Tremor, 4 to 12 Hz, bilateral postural and action tremor of the upper extremities. May also affect the head, voice, trunk, and legs. Typically is the same amplitude throughout the action, such as bringing a cup to the mouth. No other neurologic abnormalities on examination. Patients often note improvement with small amount of alcohol.

• Cerebellar tremor—an intention tremor that increases at the end of a goal-directed movement (such as finger to nose testing). Other associated neurologic abnormalities include ataxia, dysarthria, and difficulty with tandem gait. • Drug-induced—there are many drugs that enhance normal, physiologic tremor. These include caffeine, nicotine, lithium, levothyroxine, -adrenergic bronchodilators, valproate, and SSRIs. • Wilson’s Disease—wing-beating tremor that is most pronounced with shoulders abducted, elbows flexed, and fingers pointing towards each other. Usually there are other neurologic abnormalities including dysarthria, dystonia, and Kayser-Fleischer rings on ophthalmologic examination.

WORKUP • All imaging studies normal (MRI, CT) and are usually unnecessary unless there are other associated neurologic abnormalities • Check TSH

TREATMENT Do not need to treat essential tremor unless it is functionally impairing. Patients need to understand that treatments are only 40%-70% effective.

ETIOLOGY

NONPHARMACOLOGIC THERAPY

Often an inherited disease, autosomal dominant; sporadic cases without a family history are frequently encountered

Reduction of stress. Minimize use of caffeine. Small quantities of alcohol at social functions tend to be beneficial.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Parkinson’s disease—tremor is usually asymmetric, especially early on in the disease, and is predominantly a resting tremor. Patients with Parkinson’s disease will also have increased tone, decreased facial expression, slowness of movement, and shuffling gait.

ACUTE GENERAL Rx Can take a dose of propranolol (20-40 mg) in preparation for specific event.

CHRONIC Rx First-line agents • Propranolol: Usual starting dose is 30 mg. Usual therapeutic dose is 160-320 mg. Must be used with caution in those with asthma, depression, cardiac disease, and diabetes.

• Primidone: Usual starting dose is 12.5 to 25 mg hs. Usual therapeutic dose is between 62.5 and 750 mg daily. Sedation and nausea when first beginning medication are biggest side effects. Other agents • Neurontin: 400 mg qhs, usual therapeutic dose is 1200-3600 mg • Topamax: 25 mg qhs, may titrate up to about 400 mg • Alprazolam: 0.75-2.75 mg

SURGICAL Rx Thalamic deep brain stimulation contralateral to side of tremor

DISPOSITION Patients should be reassured that the condition is not associated with other neurologic disabilities; however, it can become quite functionally disabling over time.

REFERRAL This is a condition that usually can be treated by the primary care physician; however, if patient fails first-line therapies then patient should be referred to specialists for other drug trials and other possible surgical options.

PEARLS & CONSIDERATIONS Essential tremor is the most common of all movement disorders.

SUGGESTED READINGS Deuschel G, Volkmann J: Tremors: Differential diagnosis, pathophysiology, and therapy. In Jankovic J, Tolosa E (eds): Parkinson’s disease and movement disorders, ed 4, 2002, pp. 270-291. Louis ED: Essential tremor, N Engl J Med 345(12):887, 2001. Zesiewicz TA et al: Phenomenology and treatment of tremor disorders. In Hurtig H, Stern M (eds): Neurologic clinics: Movement disorders 19:3, 2001, pp. 651-680. AUTHOR: CINDY ZADIKOFF, M.D.

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Failure to Thrive BASIC INFORMATION

SYNONYMS Frailty, which could be considered a precursor or potential cause of failure to thrive, is a physiologic state of increased vulnerability to stressors, resulting from decreased physiologic reserves or dysregulation of multiple physiologic systems, clinically characterized by the presence of multiple characteristics, including weight loss, fatigue, weakness, low activity levels, slow motor performance, and balance or gait abnormalities. Sarcopenia, a decrease in muscle mass, is a common component of both failure to thrive and frailty. ICD-9CM CODES No specific codes for frailty or failure to thrive, but codes available for some of the symptoms and characteristics: 783.21 Weight loss 780.79 Fatigue 781.2 Gait abnormality 311 Depressive disorders

EPIDEMIOLOGY & DEMOGRAPHICS Because studies have not used standard definitions of either failure to thrive or frailty, estimations of incidence and prevalence are approximate and tentative. Failure to thrive affects 5% to 35% of community-dwelling older adults and 25% to 40% of nursing home residents. One study of frailty found a prevalence of 6.9% among community-dwelling older persons aged 65 years or greater, with a 4-yr incidence of 7.2%. Prevalence of both frailty and failure to thrive increases with age, and may also be higher among women than men. Although there is likely some genetic component to frailty and failure to thrive, no definitive genetic risk factors have been identified. Other potential risk factors for frailty include lower education, lower income, greater number of chronic diseases, cognitive impairment, and depression.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Criteria for failure to thrive include some combination of: • Impaired functional status • Depressive symptoms

ETIOLOGY Potential underlying causes of failure to thrive and frailty include: • Cognitive impairment • Depression • Effects of chronic or undiagnosed conditions • Sarcopenia (the loss of muscle mass and strength with age) • Immune dysregulation with a low level of chronic inflammation • Neuroendocrine dysregulation • Nutritional deficits • Elevated allostatic load (the wear and tear that occurs in an organism over time in the effort to maintain homeostasis)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Although multiple morbidity and disability often occur in persons with frailty or failure to thrive, there is evidence that these are distinct entities. Failure to thrive is a clinical syndrome, and there is no general differential diagnosis. However, it is important to evaluate for remedial causes of the components of failure to thrive (see Section II).

WORKUP Important components of the assessment of failure to thrive include: • Cognitive evaluation (e.g., MMSE) • Depressive symptoms (e.g., Geriatric Depression Scale) • Function status, including ADLs and IADLs

LABORATORY TESTS • CBC to rule out anemia or infection • Albumin and cholesterol to evaluate nutritional status • TSH to rule out hypothyroidism • Basic chemistries to evaluate electrolyte balance, renal function, and liver function • PPD to rule out tuberculosis • ESR to rule out systemic inflammatory conditions • Evaluation for potential drug toxicities or interactions (e.g., digoxin, valproic acid, Tegretol)

IMAGING STUDIES • Evaluations to rule out cancer as indicated by history and physical exam

TREATMENT There are no specific treatments for the frailty syndrome. Frail older persons may benefit from comprehensive geriatric evaluation. There are specific treatments for different components of the frailty phenotype, which are listed as follows.

NONPHARMACOLOGIC THERAPY Malnutrition: • Speech therapy evaluation and treatment as indicated • Dental evaluation and treatment • Dietary evaluation • Review and minimize dietary restrictions • Assistance with cooking or feeding Depression: • Psychotherapy • Increased socialization

DISEASES AND DISORDERS

Failure to thrive is a state of decline in an older person that is multifactorial, manifested by weight loss, decreased appetite, poor nutrition, and inactivity, often accompanied by dehydration, depressive symptoms, impaired immune function, and low cholesterol.

• Nutritional status • Medication review (medications that can contribute to failure to thrive include anticholinergics, antiepileptics, digoxin, -blockers, benzodiazepines, neuroleptics, antidepressants, and glucocorticoids) • Evaluation of chronic diseases that contribute to failure to thrive (e.g., cancer, chronic lung disease, heart failure, diabetes, recurrent infections, rheumatologic diseases, dementia, stroke, TB, and other systemic infections) There are no specific laboratory tests or imaging studies generally indicated for evaluation of frailty or failure to thrive. Some basic tests that may be considered to rule out potentially reversible conditions that may contribute to these syndromes are listed below. This list is not exhaustive, and evaluation should be guided by the findings on history and physical.

Section I

DEFINITION

• Cognitive impairment • Poor nutrition or weight loss Multiple sets of criteria have been proposed for defining frailty, including: • Low physical activity levels with weight loss • Three or more of the following: unintentional weight loss, fatigue/exhaustion, weak grip strength, slow gait speed, low physical activity Elements of the history consistent with failure to thrive or frailty: • Fatigue or generalized weakness • Weight loss • Falls • Low levels of physical activity • Depressive symptoms • Functional decline or impairment in I/ADLs Physical findings consistent with failure to thrive or frailty: • Slow gait speed (1 m/sec) • Abnormalities of gait or balance • Weakness (poor grip strength) • Low BMI (e.g., 22) • Cognitive impairment • Decubitus ulcers

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Failure to Thrive Functional impairment: • Resistance training (improve muscle mass and strength) • Physical therapy (improve gait and balance) • Occupational therapy (improve ADLs) • Increased physical activity

ACUTE GENERAL Rx • Failure to thrive and frailty are chronic conditions, with no acute treatments.

CHRONIC Rx • Depression: antidepressants • Cognitive impairment: cholinesterase inhibitors, memantine • Malnutrition: nutritional supplements, can consider appetite-augmenting medications such as mirtazapine, megestrol, or marinol

DISPOSITION • Older persons with frailty or failure to thrive are at high risk for adverse events such as further functional decline and death, as well as high health care utilization and costs and need for services such as home health care, assisted living, or skilled nursing facilities.

REFERRAL • Referral for comprehensive geriatrics assessment, physical therapy, occupational therapy, dietary evaluation, or speech therapy is often appropriate.

• Social work referral for assistance accessing various community services can be beneficial, especially when disability is present.

PEARLS & CONSIDERATIONS COMMENTS • Failure to thrive and frailty are concepts under development, with no standard definitions. • Research into the underlying mechanisms and potential interventions for failure to thrive and frailty is ongoing.

PREVENTION • Early diagnosis and treatment of depression, cognitive impairment, and malnutrition may help prevent or delay failure to thrive. • Although there are currently no preventive interventions for frailty with a strong evidence base, there is fair evidence that increased physical activity, particularly with a component of resistance training, can improve or prevent some of the characteristics associated with frailty and potentially decrease functional decline.

PATIENT/FAMILY EDUCATION • Frail older adults and their caregivers can benefit from referral to community services as appropriate.

• Failure to thrive is often a prelude to death in older persons, and presence of failure to thrive should trigger discussions of goals of care and advanced directives with the patient and family. • If evaluation and treatment of the components of failure to thrive does not improve the patient’s condition, the options of hospice or palliative care should be discussed. • There is no evidence that artificial feeding or hydration prolong life or relieve suffering in the setting of failure to thrive.

SUGGESTED READINGS Ferrucci L et al: Designing randomized, controlled trials aimed at preventing or delaying functional decline and disability in frail, older persons: a consensus report, J Am Geriatr Soc 52:625-634, 2004. Fried LP et al: Frailty in older adults: evidence for a phenotype, J Gerontol A Biol Sci Med Sci 56:M146-M157, 2001. Fried LP et al: Untangling the concepts of disability, frailty, and comorbidity: implications for improved targeting and care, J Gerontol A Biol Sci Med Sci 59:M255-M263, 2004. Hogan DB et al: Models, definitions, and criteria of frailty, Aging Clin Exp Res 15(suppl 3):1-29, 2003. Robertson RG, Montagni M: Geriatric failure to thrive, Am Fam Physician 70(2):343-350, 2004. Sarkisian CA, Lachs MS: “Failure to thrive” in older adults, Ann Int Med 124(12):10721078, 1996. AUTHOR: SUSAN E. HARDY, M.D., PH.D.

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Falls BASIC INFORMATION

ICD-9CM CODES E880.1 Accidental fall on or from sidewalk E880.9 Accidental fall on or from other stairs or steps E885.9 Fall from other slipping, tripping, or stumbling, fall on moving sidewalk E888.1 Fall resulting in striking against other object E888.8 Other fall E888.9 Unspecified fall 781.2 Abnormality of gait: ataxic, paralytic, spastic, staggering

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: Estimated at one in three people aged 65 or older, living in the community. One in two nursing home dwelling elders. The national nursing home fall rate is 1.6 per bed per year. One in 10 falls results in a serious injury. RISK FACTORS: Independent risk factors for falls include: • Arthritis • Depressive symptoms • Orthostasis • Impairment in cognition, vision, balance, gait, or muscle strength • Use of four or more prescription medications RECURRENCE RATE: Approximately 50% in a given year

PHYSICAL FINDINGS & CLINICAL PRESENTATION May present to the emergency department with injury. Falls may not be a presenting complaint in the geriatrics population; however, due to the frequency of falls in elders, an assessment of fall risk should be integrated into the history and physical examination in all geriatrics patients regardless of whether they are seen for falls or not. History: • History of prior falls  Frequency/pattern of falls • Cause of the fall  Activity at the time of the fall  Environment surrounding the fall (e.g., obstacles, uneven/slippery surface) • Symptoms preceding the fall  Weakness, dizziness, palpitations, loss of consciousness

ETIOLOGY The causes of falls are complex but typically involve the interaction between the environment and multiple predisposing factors that increase in prevalence with age such as: • Changes that affect gait and balance:  Decreased proprioception  Increased postural sway  Orthostatic hypotension  Slower righting reflexes • Medical conditions that can affect stability of gait:  Osteoarthritis  Stroke  Peripheral neuropathy • Sensory impairment in vision and hearing. • Medications associated with falls are cardiovascular medications that cause orthostatic hypotension, sedatives, or psychoactive medications that can affect gait and balance.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Near fall • Syncope

• As dictated by history and physical examination • Drug levels to diagnose specific medication toxicities where suspected

IMAGING STUDIES • For the diagnosis or exclusion of fracture • Neuroimaging to rule out intracranial process if focal neurologic findings present on physical examination

TREATMENT ACUTE GENERAL Rx • Treatment of injury resulting from the fall

CHRONIC Rx Interventions to reduce future falls: • Optimization of chronic conditions that may have contributed to the fall • Reduction of medications with potential sedative and orthostatic effects • Reduction of home environmental hazards • Minimization of activities with high risk for injury • Appropriate footwear with low heel and thin sole to maximize proprioceptive input • Teach compensatory strategies for postural hypotension • Assess for need of an assist device (cane, walker, etc.) for ambulation • Physical therapy for strengthening, range of motion, gait and balance training Interventions to reduce injurious falls: • Assess for coexistent osteoporosis and treat to ensure proper bone health. • Consider hip protectors in motivated individuals.

DISPOSITION • Due to the high recurrence rate of falls, older adults with a history of falls require longitudinal assessments with regular re-evaluation.

REFERRAL • Where abnormalities of strength, gait, or balance are detected, referral to physical therapy for gait, balance, and strength training • To home health agency for home environmental hazard assessment • In frail elders or in recurrent falls, referral for a comprehensive geriatrics assessment

DISEASES AND DISORDERS

A fall is defined as an unintentional change in position resulting in an individual coming to rest at a lower level. They typically result from multiple risk factors, changes in gait and balance, and chronic disease in elders.

LABORATORY TESTS Section I

DEFINITION

• Symptoms following the fall  Injury  Unable to stand without assistance (long lie) • Use of walking aids—cane/walker • Detailed medical history  Current medical diagnoses  Medication use  Alcohol consumption • Home environment—lighting, stairs, loose rugs, bathroom, showers, etc. • Inquire into fear of falling and restrictions in daily activities due to fear of falling Physical examination: • Assessment of vision • Measurement of postural blood pressure • Neurologic examination for proprioception, muscle strength • Assessment of cognitive function • Musculoskeletal examination of the legs and feet • Cardiovascular examination for dysrhythmia and potential syncope • Examine for gait and balance disorders. The Get Up and Go test:  Rise from a straight-backed chair.  Walk 10 feet, using usual walking aid (cane, walker, etc.).  Turn.  Return to the chair and sit down.  Observe for: 1. Balance with sitting and upon standing 2. Pace and stability with walking 3. Ability to turn without staggering 4. Time to complete task

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Falls PEARLS & CONSIDERATIONS COMMENTS • Although common in older adults, gait instability and falls are not a part of normal aging. • Falls are typically a manifestation of underlying comorbidity and frailty and it is these underlying causes that need to be addressed to prevent future adverse health outcomes.

PREVENTION • Early identification of high-risk individuals offers greatest promise for fall prevention.

• Due to the frequency of falls, an assessment of fall risk should be integrated into the evaluation of all geriatrics patients regardless of whether they are seen for falls or not. • Intervention should be directed at the identified underlying cause.

PATIENT/FAMILY EDUCATION • Falls are not a manifestation of normal aging. • It is possible to decrease fall risk with a multifaceted intervention; however, an elder who has fallen will remain at risk for future falls.

SUGGESTED READINGS Bloem BR et al: Falls in the elderly I. Identification of risk factors. Wien Klin Wochenschr 113(10):352-362, 2001. Boers I et al: Falls in the elderly II. Strategies for prevention. Wien Klin Wochenschr 113(11-12):398-407, 2001. Tinetti ME: Preventing falls in elderly persons, N Engl J Med 348(1):42-48, 2003. AUTHOR: RAM MILLER, M.D.C.M., M.S., F.R.C.P.C.

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Femoral Neck Fracture TREATMENT

DEFINITION

• Orthopedic consultation • Surgery indicated in most cases, usually within 24 hr • DVT prophylaxis

A femoral neck fracture occurs within the capsule of the hip joint between the base of the head and the intertrochanteric line. Intracapsular fracture Subcapital fracture ICD-9CM CODES 820.8 Femoral neck fracture

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Lifetime risk in women approximately 16% PREVALENT SEX: Female:male ratio of 3:1 PREVALENT AGE: 90% over age 60

PHYSICAL FINDINGS & CLINICAL PRESENTATION • A hip or groin pain • Affected limb usually shortened and externally rotated in displaced fractures • Impacted fractures: possibly no deformity and only mild pain with hip motion • Mild external bruising

ETIOLOGY • Trauma • Age-related bone weakness, usually caused by osteoporosis • Increased risk of fractures in elderly (decline in muscle function, use of psychotropic medication, etc.)

DIAGNOSIS

DISPOSITION • Mortality rate within 1 yr in elderly patients is 25% to 30%. • Dementia is a particularly poor prognostic sign.

PEARLS & CONSIDERATIONS COMMENTS • Complications: nonunion and avascular necrosis • Intracapsular fractures: occasionally occur in nonambulatory patients 1. Usually treated nonsurgically, especially in the patient with dementia and limited pain perception 2. Early bed-to-chair mobilization and vigilant nursing care to avoid skin breakdown 3. Fracture usually pain free in a short time even if solid bony healing does not occur • As a result of the increasing life span of the female population, femoral neck fractures are becoming more common. The initial physical examination and roentgenographic studies may be completely negative. Groin pain, sometimes quite severe, may be the only early clue to the diagnosis.

SUGGESTED READINGS Bettelli G et al: Relationship between mortality and proximal femur fractures in the elderly, Orthopedics 26:1045, 2003. Feldstein AC et al: Older women with fractures: patients falling through the cracks of guideline recommended osteoporosis screening and treatment, J Bone Joint Surg 85A:2294, 2003. Jain R et al: Comparison of early and delayed fixation of subcapital hip fractures in patients sixty years of age or less, Bone Joint Surg 84(A):1605, 2002. Kaufman JD et al: Barriers and solutions to osteoporosis care in patients with a hip fracture, J Bone Joint Surg 85A:1837, 2003. Lawrence VA et al: Medical complications and outcomes after hip fracture repair, Ann Intern Med 162:2053, 2003. McClung MR et al: Effect of risedronate on the risk of hip fracture in elderly women. Hip Intervention Program Study Group, N Engl J Med 344(5):333, 2001. McKinley JC, Robinson CM: Treatment of displaced intracapsular fractures with total hip arthroplasty, J Bone Joint Surg 84(A):2010, 2002. Schoofs MW et al: Thiazide diuretics and the risk for hip fractures, Ann Intern Med 139:476, 2003. Stevens JA, Olson S: Reducing falls and resulting hip fractures among older women, Home Care Prov 5(4):134, 2000. AUTHOR: LONNIE R. MERCIER, M.D.

DIFFERENTIAL DIAGNOSIS Osteoarthritis of hip Pathologic fracture Lumbar disc syndrome with radicular pain Insufficiency fracture of pelvis

WORKUP Diagnosis usually obvious based on clinical and radiographic findings

IMAGING STUDIES • Standard roentgenograms consisting of an AP of the pelvis and a cross-table lateral of the hip to confirm the diagnosis (Fig. 1-18) • If initial roentgenograms negative and diagnosis of an occult femoral neck fracture suspected, hospital admission and further radiographic assessment with either bone scanning or MRI • Bone scanning most sensitive after 48 to 72 hr.

FIGURE 1-18 Femoral neck fracture. (From Scudieri G [ed]: Sports medicine: principles of primary care, St Louis, 1997, Mosby.)

DISEASES AND DISORDERS

SYNONYMS

• The rate of hip fracture could be reduced by: 1. Elimination of environmental hazards (poor lighting, loose rugs) 2. Regular exercise for balance and strength 3. Patient education about fall prevention 4. Medication review to minimize side effects 5. Prevention and treatment of osteoporosis

Section I

BASIC INFORMATION

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Fibromyalgia BASIC INFORMATION

DIAGNOSIS

TREATMENT

DEFINITION

DIFFERENTIAL DIAGNOSIS

ACUTE GENERAL Rx

Fibromyalgia is a poorly defined disorder characterized by multiple trigger points and referred pain.

• • • • • • • •

• Self-management • Explanation, reassurance • Vit D replacement in vit D deficient patients • Aerobic and stretching exercise, particularly swimming • Mild analgesics; avoidance of chronic narcotic use • Trigger point injections • Physical therapy • SSRIs if associated depression

SYNONYMS Myofascial pain syndrome Fibrositis Psychogenic rheumatism Nonarticular rheumatism Fibromyalgia syndrome (FS) ICD-9CM CODES 729.0 Rheumatism, unspecified and fibrositis 729.1 Myalgia and myositis, unspecified

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 1% to 2% of the general population PREVALENT SEX: Female:male ratio of 9:1

PHYSICAL FINDINGS Tender “nodules” and tender points (Fig. 1-19)

ETIOLOGY • Unknown • Pain magnification may play a role • Associated mood disorder is common

Polymyalgia rheumatica Referred discogenic spine pain Rheumatoid arthritis Localized tendinitis Connective tissue disease Osteoarthritis Thyroid disease Spondyloarthropathies

WORKUP • Subsets of this disorder are often described: 1. If symptoms develop in conjunction with other conditions (rheumatoid disease or acute stress) 2. If findings are more regionally distributed, such as those in the neck following motor vehicle accidents • The primary condition is often suggested by the following criteria from the American College of Rheumatology: 1. History of widespread pain 2. Pain in 11 of 18 selected tender spots on digital palpation (mainly in the spine, elbows, and knees)

LABORATORY TESTS There are no abnormalities in fibromyalgia, but laboratory assessment may be required to rule out other conditions and may include: • CBC, ESR, rheumatoid factor, ANA • CPK, T4 • 25-hydroxyvitamin D

FIGURE 1-19 The sites of the 18 tender points of the 1990 ACR criteria for the classification of fibromyalgia. (From Conn R: Current Diagnosis, ed 9, Philadelphia, 1997, WB Saunders.)

DISPOSITION • Prognosis is uncertain. • Symptoms come and go for years in spite of an aggressive multifaceted approach to treatment.

PEARLS & CONSIDERATIONS COMMENTS • Before making this diagnosis, all other more likely disorders should be ruled out. • The term “fibrositis” is often used, but no inflammation has ever been found. • The number of trigger points needed to establish the diagnosis is debated.

SUGGESTED READINGS Clauw DJ: Elusive syndromes: treating the biologic basis of fibromyalgia and related syndromes, Cleve Clin J Med 68:830, 2001. Crofford LJ: Pharmaceutical treatment options for fibromyalgia, Curr Rheumatol Rep 6:274, 2004. Gracely RH et al: Functional magnetic resonance imaging evidence of augmented pain processing in fibromyalgia, Arthritis Rheum 46:1333, 2002. Hakkinen A et al: Strength training induced adaptations in neuromuscular function of premenopausal women with fibromyalgia: comparison with healthy women, Ann Rheum Dis 60:21, 2001. Richards SCM, Scott DL: Prescribed exercise in people with fibromyalgia: parallel group randomized controlled trial, BMJ 325:185, 2002. Robinson RL et al: Depression and fibromyalgia: treatment and cost when diagnosed separately or concurrently, J Rheumatol 31:1621, 2004. Worrel LM et al: Treating fibromyalgia with a brief interdisciplinary program: initial outcomes and predictors of response, Mayo Clin Proc 76:381, 2001. AUTHOR: LONNIE R. MERCIER, M.D.

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Gastric Cancer BASIC INFORMATION Gastric cancer is an adenocarcinoma arising from the stomach.

SYNONYMS

ICD-9CM CODES 451 Malignant neoplasm of stomach

EPIDEMIOLOGY & DEMOGRAPHICS • Annual incidence of gastric cancer in the U.S. is 7 cases/100,000 persons. The incidence is much higher in Japan, with rates as high as 80 cases/100,000 persons. • Most gastric cancers arise in the antrum (35%). • The incidence of distal stomach tumors has greatly declined whereas that of proximal tumors of the cardia and fundus is on the rise. • Gastric cancer occurs most commonly in male patients 65 yr (70% of patients are 50 yr). • Incidence of gastric cancer has been declining over the past 30 yr. • Male:female ratio is 3:2. • Familial diffuse gastric cancer is a disease with autosomal dominant inheritance in which gastric cancer develops at a young age. Germ-line truncating mutations in the E-cadherin gene (CDH1) is found in these families.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Medical history may reveal complaints of postprandial fullness with significant weight loss (70% to 80%), nausea/emesis (20% to 40%), dysphagia (20%), and dyspepsia, usually unrelieved by antacids; epigastric discomfort, usually lessened by fasting and exacerbated by food intake, is also common. • Epigastric or abdominal mass (30% to 50%), epigastric pain. • Skin pallor secondary to anemia. • Hard, nodular liver: generally indicates metastatic disease to the liver. • Hemoccult-positive stools. • Ascites, lymphadenopathy, or pleural effusions: may indicate metastasis.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Gastric lymphoma (5% of gastric malignancies) • Hypertrophic gastritis • Peptic ulcer • Reflux esophagitis

TREATMENT ACUTE GENERAL Rx • Gastrectomy with regional lymphadenectomy is performed in patients with curative potential (30% of patients at time of diagnosis). Post-op adjuvant chemoradiotherapy using 5-fluorouracil and leucovorin is now the standard of care for resected patients able to tolerate such treatment. Postoperative chemotherapy and radiotherapy, compared with surgical resection alone, can extend the survival of patients with gastric cancer in those who are able to complete adjuvant therapy. • When surgical cure is not possible, palliative resection may prolong duration and quality of life. • Chemotherapy (FAM: 5-fluorouracil, Adriamycin, and mitomycin C) may provide some palliation; however, it generally does not prolong survival. Chemotherapy with docetaxel, cisplatin, and 5-fluorouracil can be used for chemotherapy-naive patients with metastatic or locally recurrent gastric cancer.

DISPOSITION

Upper endoscopy with biopsy will confirm diagnosis. Endoscopic ultrasonography in combination with CT scanning and operative lymph node dissection can be used in staging of the tumor.

• 5-yr survival rate of gastric carcinoma is 12% overall. • 5-yr survival for early gastric cancers (usually detected incidentally with endoscopy in populations where screening is recommended) is 35%.

LABORATORY TESTS

REFERRAL

• • • •

Surgical referral for resection

WORKUP

Microcytic anemia Hemoccult-positive stools Hypoalbuminemia Abnormal liver enzymes in patients with metastasis to the liver • Mutation-specific predictive genetic testing by PCR amplification followed by restriction—enzyme digestion and DNA sequencing for truncating mutations in the E-cadherin gene (CDH1) is recommended in families of patients with familial diffuse cancer because gastric cancer develops in three of every four carriers of a mutant CDH1 gene.

IMAGING STUDIES • Upper GI series with air contrast (90% accurate) should be considered only if endoscopy is not readily available • Abdominal CT scan to evaluate for metastasis (70% accurate for regional node metastases)

PEARLS & CONSIDERATIONS COMMENTS • Gastrectomy patients will need vitamin B12 replacement. They are also at risk for dumping syndrome and should be advised to ingest frequent, small meals. • Prophylactic gastrectomy should be considered in young asymptomatic carriers of germ-line truncating CDH1 mutations who belong to families with highly penetrant heredity diffuse gastric cancer.

SUGGESTED READINGS Layke J, Lopez P: Gastric cancer: Diagnosis and treatment options, Am Fam Physician 69:1133, 2004. McDonald JS: Chemotherapy in the management of gastric cancer, JCO 21:2768, 2003. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Stomach cancer Linitis plastica

Risk factors: • Chronic H. pylori gastritis. Gastric cancer develops in persons infected with H. pylori. Those with histologic findings of severe gastric atrophy, corpus-predominant gastritis, or intestinal metaplasia are at increased risk. Persons with H. pylori infection and duodenal ulcer are not at risk, whereas those with gastric ulcers, nonulcer dyspepsia, and gastric hyperplastic polyps are. • Tobacco abuse, alcohol consumption • Food additives (nitrosamines), smoked foods, occupational exposure to heavy metals, rubber, asbestos • Chronic atrophic gastritis with intestinal metaplasia, hypertrophic gastritis, and pernicious anemia

Section I

DEFINITION

ETIOLOGY

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Gastroesophageal Reflux Disease BASIC INFORMATION DEFINITION Gastroesophageal reflux disease (GERD) is a motility disorder characterized primarily by heartburn and caused by the reflux of gastric contents into the esophagus.

SYNONYMS Reflux esophagitis GERD ICD-9CM CODES 530.81 Gastroesophageal reflux disease 530.1 Esophagitis 787.1 Heartburn

EPIDEMIOLOGY & DEMOGRAPHICS GERD is one of the most prevalent GI disorders. Nearly 7% of persons in the U.S. experience heartburn daily, 20% experience it monthly, and 60% experience it intermittently. Nearly 20% of adults use antacids or OTC H2-blockers at least once a week for relief of heartburn.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Physical examination: generally unremarkable • Clinical signs and symptoms: heartburn, dysphagia, sour taste, regurgitation of gastric contents into the mouth • Chronic cough and bronchospasm • Chest pain, laryngitis, early satiety, abdominal fullness, and bloating with belching

ETIOLOGY • Incompetent lower esophageal sphincter (LES) • Medications that lower LES pressure (calcium channel blockers, -adrenergic blockers, theophylline, anticholinergics) • Foods that lower LES pressure (chocolate, yellow onions, peppermint) • Tobacco abuse, alcohol, coffee • Obesity • Gastric acid hypersecretion • Hiatal hernia (controversial) present in 70% of patients with GERD; however, most patients with hiatal hernia are asymptomatic

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Peptic ulcer disease • Angina • Esophagitis (from infections such as herpes, Candida), medication induced (doxycycline, potassium chloride) • Esophageal spasm (nutcracker esophagus) • Cancer of esophagus

WORKUP Aimed at eliminating the conditions noted in the differential diagnosis and documenting the type and extent of tissue damage

LABORATORY TESTS • 24-hr esophageal pH monitoring and Bernstein test are sensitive diagnostic tests. They are useful in patients with atypical manifestations of GERD, such as chest pain or chronic cough. • Esophageal manometry is indicated in patients with refractory reflux in whom surgical therapy is planned. • Upper GI endoscopy (EGD) is useful to document the type and extent of tissue damage in GERD and to exclude Barrett’s esophagus. The American College of Gastroenterology recommends endoscopy to screen for Barrett’s esophagus in patients who have chronic GERD symptoms. Barrett’s esophagus is a precursor to esophageal cancer. Once diagnosed, yearly surveillance (EGD) is appropriate. Once excluded, EGD need not be repeated unless new symptoms develop (e.g., dysphagia).

IMAGING STUDIES • Upper GI series can identify ulcerations and strictures; however, it may miss mucosal abnormalities. Only one third of patients with GERD have radiographic signs of esophagitis.

TREATMENT NONPHARMACOLOGIC THERAPY • Lifestyle modifications with avoidance of foods (citrus- and tomato-based products, chocolate) and drugs that exacerbate reflux (e.g., caffeine, blockers, calcium channel blockers, alpha-adrenergic agonists, theophylline) • Avoidance of tobacco and alcohol use • Elevation of head of bed 4 to 8 inches using blocks • Avoidance of lying down directly after late or large evening meals • Weight reduction, decreased fat intake • Avoid clothing tight around the waist

ACUTE GENERAL Rx • Proton pump inhibitors (PPIs) (esomeprazole 40 mg qd, omeprazole 20 mg qd, lansoprazole 30 mg qd, rabeprazole 20 mg qd, or pantoprazole 40 mg qd) are safe, tolerated, and very effective in most patients. • H2-blockers (nizatidine 300 mg qhs, famotidine 40 mg qhs, ranitidine 300 mg qhs, or cimetidine 800 mg qhs) can be used but are generally much less effective than PPIs. • Antacids (may be useful for relief of mild symptoms; however, they are

generally ineffective in severe cases of reflux). • Prokinetic agents (metoclopramide) are indicated only when PPIs are not fully effective. They can be used in combination therapy; however, side effects limit their use. • For refractory cases: surgery with Nissen fundoplication. Potential surgical candidates should have reflux esophagitis documented by EGD and normal esophageal motility as evaluated by manometry. Surgery generally consists of reduction of hiatal hernia when present and placement of a gastric wrap around the GE junction (fundoplication). Although laparoscopic fundoplication is now widely used, surgery should not be advised with the expectation that patients with GERD will no longer need to take antisecretory medications or that the procedure will prevent esophageal cancer among those with GERD and Barrett’s esophagus. • Endoscopic radiofrequency heating of the GE junction (Stretta procedure) is a newer treatment modality for GERD patients unresponsive to traditional therapy. Endoscopic gastroplasty (EndoCinch procedure) also aims at treating GERD.

DISPOSITION • The majority of the patients respond well to therapy. • Recurrence of reflux is common if treatment is discontinued. • Postsurgical complications occur in nearly 20% of patients (dysphagia, gas bloating, diarrhea, nausea). Long-term follow-up studies also reveal that within 3 to 5 years 52% of patients who had undergone antireflux surgery are taking antireflux medications again.

REFERRAL • GI referral for upper endoscopy is needed when there are concerns about associated PUD, Barrett’s esophagus, or esophageal cancer. • Patients with Barrett’s esophagus should undergo surveillance endoscopy with mucosal biopsy every 2 yr or less because the risk of developing adenocarcinoma of esophagus is at least 30 times greater than that of the general population.

SUGGESTED READINGS Heidelbaugh JL et al: Management of gastroesophageal reflux disease, Am Fam Physician 68:1311, 2003. Shaheen N, Ransohoff DF: Gastroesophageal reflux, Barrett esophagus, and esophageal cancer, JAMA 287:1972, 2002. AUTHORS: FRED F. FERRI, M.D., with revisions by TOM J. WACHTEL, M.D.

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Giant Cell Arteritis BASIC INFORMATION

SYNONYMS Temporal arteritis Cranial arteritis Horton’s disease ICD-9CM CODES 446.5 Temporal arteritis

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 200 cases/100,000 persons; female-to-male predominance of two- to four-fold INCIDENCE: 17 to 23.3 new cases/ 100,000 persons 50 yr

CLINICAL PRESENTATION & PHYSICAL FINDINGS GCA can present with the following clinical manifestations: • Headache, often associated with marked scalp tenderness • Constitutional symptoms (fever, weight loss, anorexia, fatigue) • Polymyalgia syndrome (aching and stiffness of the trunk and proximal muscle groups) • Visual disturbances (transient or permanent monocular visual loss) • Intermittent claudication of jaw and tongue on mastication Important physical findings in GCA: • Vascular examination: Tenderness, decreased pulsation, and nodulation of temporal arteries; diminished or absent pulses in upper extremities

ETIOLOGY Vasculitis of unknown etiology

DIAGNOSIS Clinical history and vascular examination are cornerstones of diagnosis. The presence of any three of the following five items allows the diagnosis of GCA with a sensitivity of 94% and a specificity of 91%: • Age of onset 50 yr • New-onset or new type of headache • Temporal artery tenderness or decreased pulsation on physical examination • Westergren ESR 50 mm/hr

DIFFERENTIAL DIAGNOSIS • Other vasculitic syndromes • Nonarteritic Anterior Ischemic Optic Neuropathy (AION) • Primary amyloidosis • TIA, stroke • Infections • Occult neoplasm, multiple myeloma

REFERRAL

WORKUP

PEARLS & CONSIDERATIONS

LABORATORY TESTS • ESR 50 mm/hr; however, up to 22.5% patients with GCA have normal ESR before treatment • C-reactive protein is typically included in lab investigation; it has greater sensitivity than ESR • Mild to moderate normochromic normocytic anemia, elevated platelet count • IL-6 levels hold promise for a more sensitive modality, but at this stage remains experimental

IMAGING STUDIES • Reliability of color duplex ultrasonography of temporal artery is controversial as it is thought that it does not improve diagnostic accuracy over careful physical examination • Fluorescein angiogram of ophthalmic vessels may be warranted to differentiate between arteritic AION (i.e., GCA) and nonarteritic AION

TREATMENT ACUTE GENERAL Rx • Intravenous methylprednisolone (5001000 mg qd for 3-5 days) is indicated in those with significant clinical manifestations (e.g., visual loss). • Oral prednisone (1 mg/kg/day) may be used under less urgent circumstances or following the initial period of treatment with intravenous methylprednisolone. High-dose oral regimen should be continued at least until symptoms resolve and ESR returns to normal. • Prednisone should be tapered gradually (~5 mg every other wk) initially and subsequently even more slowly (2.5 mg every 2-4 wk). Steroid treatment is usually required for at least 6 mo, and sometimes as much as 2 yr. • Methotrexate or azathioprine may be added to the steroid regimen for their steroid-sparing effect, but efficacy is unproven.

DISPOSITION If steroid therapy is initiated early, GCA has excellent prognosis; however, 20% of patients have permanent partial or com-

• Surgical referral for biopsy of temporal artery • Ophthalmology referral in patients with visual disturbances and following initiation of corticosteroid therapy • Rheumatology referral for difficult cases

COMMENTS • The relationship between polymyalgia rheumatica and GCA is unclear, but the two may frequently coexist. • Clinical picture rather than ESR should be the prime yardstick for continuing prednisone therapy. • A rising ESR in a clinically asymptomatic patient with normal hematocrit should raise suspicion for alternate explanations (e.g., infections, neoplasms). • Although pathologic findings on temporal artery biopsy are the gold standard for the diagnosis of GCA, the false-negative rate is around 9% (the false-negative rate may be lower in the hands of an experienced surgeon); in some cases, a second biopsy from the contralateral side may be required. • GCA is associated with a markedly increased risk for the development of aortic aneurysm, which is often a late complication and may cause death. Annual chest radiograph in chronic CGA patients has been suggested, as well as emergent chest CT or MRI for clinical suspicion.

SUGGESTED READINGS Please refer to references within these papers as well as associated ‘Letters to the Editor’ for further details. Gold R et al: Therapy of neurological disorders in systemic vasculitis, Sem Neurol 23(2):207, 2003. Hayreh SR et al: Visual improvement with corticosteroid therapy in giant cell arteritis. Report of large study and review of the literature, Acta Ophthalmol Scand 80:355, 2002. Hoffman GS et al: A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate for giant-cell arteritis, Arthritis and Rheum 46(5):1309, 2002. Norborg E, Norborg C: Giant cell arteritis: epidemiological clues to its pathogenesis and an update on its treatment, Rheumatol 42:413, 2003. Salvarani C et al: Polymyalgia rheumatica and giant-cell arteritis, N Engl J Med 347(4):261, 2002. Smetana GW, Shmerling RH: Does this patient have temporal arteritis? JAMA 287:92, 2002. AUTHOR: U. SHIVRAJ SOHUR, M.D, PH.D.

DISEASES AND DISORDERS

Giant cell arteritis (GCA) is a segmental systemic granulomatous arteritis affecting medium- and large-sized arteries in individuals 50 years. Inflammation primarily targets extracranial blood vessels, and although the carotid system is usually affected, pathology in posterior cerebral artery has been reported.

plete loss of vision. Once there is visual loss, improvement is dismal: in one study, only 4% of eyes improved in both visual acuity and central visual field.

Section I

DEFINITION

• Temporal artery biopsy with vasculitis and mononuclear cell infiltrate or granulomatous changes

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Glaucoma, Chronic Open-Angle BASIC INFORMATION DEFINITION Chronic open-angle glaucoma refers to optic nerve damage often associated with elevated intraocular pressure; it is a chronic, slowly progressive, usually bilateral disorder associated with visual loss, eye pain, and optic nerve damage. Now felt to be a primary disease of the optic nerve with high pressure a high risk factor for glaucoma.

SYNONYM Chronic simple glaucoma ICD-9CM CODES 365.1 Open-angle glaucoma

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): Third most common cause of visual loss (75% to 95% of all glaucomas are open angle.) PREVALENCE (IN U.S.): • Overall prevalence in U.S. population 40 yr of age is estimated to be 1.86%, with 1.57 million white and 398,000 black patients affected. • 150,000 patients suffer bilateral blindness. • Disease occurs in 2% of people 40 yr old. • Prevalence is higher in diabetics, with high myopia, and among older persons. • More common in blacks (3  the ageadjusted prevalence than whites). PREDOMINANT AGE: • Persons 50 yr old PEAK INCIDENCE: • Increases after 40 yr • Because of rapid aging of the U.S. population, expect 3 million cases by year 2020. GENETICS: • Four to six times higher incidence in blacks than whites • No clear-cut hereditary patterns but a strong hereditary tendency

PHYSICAL FINDINGS • High intraocular pressures and large optic nerve cup (Ocular Hypertension Treatment Study—very important)

• Cornea thickens faster in vision loss • Abnormal visual fields • Open-angle gonioscopy •`Red eye • Restricted vision and field

ETIOLOGY • • • • •

Uncertain hereditary tendency Topical steroids Trauma Inflammatory High-dose oral corticosteroids taken for prolonged periods

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Other optic neuropathies • Secondary glaucoma from inflammation and steroid therapy • Red eye differential • Trauma • Contact lens injury

WORKUP • • • • • •

Intraocular pressure Slit lamp examination Visual fields Gonioscopy Nerve fiber analysis—GDx Corneal thickness—very important in prognosis

LABORATORY TESTS Blood sugar

IMAGING STUDIES • Optic nerve photography—stereo photographs • Visual field testing • GDx (laser scan of nerve fiber layer)

TREATMENT ACUTE GENERAL Rx • -Blockers (Timolol) qd to bid depending on individual response to drug • Diamox 250 mg qid or pilocarpine • Hyperosmotic agents (mannitol) in acute treatment • Prostaglandins • Laser trabeculoplasty (SLT) as needed • Pilocarpine qid

CHRONIC Rx • At least biannual checks of intraocular pressure and adjustment of medication • Poor control = frequent examinations; good control = drugs • Trabectalectomy • Filter valves

DISPOSITION Must be followed by ophthalmologist

REFERRAL Immediately to ophthalmologist

PEARLS & CONSIDERATIONS COMMENTS • Glaucoma is a serious blinding disease. Must be followed professionally by an ophthalmologist. • Early diagnosis and treatment may minimize visual loss. • Glaucoma is not solely caused by increased intraocular pressure, because approximately 20% of patients with glaucoma have normal intraocular pressure, but high pressure is definitely a risk factor to be considered.

SUGGESTED READINGS Gordon MO et al: Baseline factors that predict the onset of primary open-angle glaucoma, Arch Ophthalmol 120:714, 2002. Heijl A et al: Reduction of intraocular pressure and glaucoma progression: Results from the early manifest glaucoma trial, Arch Ophthalmol 120:1268, 2002. Higginbotham EJ et al: The Ocular Hypertension Treatment Study: topical medication delays or prevents primary open-angle glaucoma in African American individuals, Arch Ophthalmol 122(6):813, 2004. Rezaie T et al: Adult-onset primary open-angle glaucoma caused by mutations in optineurin, Science 295:1077, 2002. AUTHOR: MELVYN KOBY, M.D.

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Glaucoma, Primary Closed-Angle BASIC INFORMATION

ETIOLOGY • Narrow angles with acute closure— blockage of circulatory path of the aqueous humor causing increase in interior ocular pressure

SYNONYMS

DIAGNOSIS

Acute glaucoma Pupillary block glaucoma Narrow-angle glaucoma

DIFFERENTIAL DIAGNOSIS

ICD-9CM CODES 365.2 Primary angle-closure glaucoma

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • In 2% to 8% of all patients with glaucoma • Higher incidence among those with hyperopia, small eyes, dense cataracts, shallow anterior chambers PREDOMINANT SEX: Females  males PREDOMINANT AGE: 50 to 60 yr PEAK INCIDENCE: Greater after 50 yr of age; high association with hypopia, cataracts, and eye trauma GENETICS: High family history

PHYSICAL FINDINGS & CLINICAL FINDINGS • • • • • • •

Hazy cornea (Fig. 1-20) Narrow angle Red eyes Pain Injection of conjunctiva Shallow anterior chamber Thick cataract

• Fundus photography • Fluorescein angiography for neurovascular disease

TREATMENT The goal of treatment is to acutely lower pressure on eye and keep it down.

NONPHARMACOLOGIC THERAPY • • • • • • • • • • •

Laser iridotomy early in disease process

High pressure Optic nerve cupping Field loss Shallow chamber Open-angle glaucoma Conjunctivitis Corneal disease-keratitis Uveitis Scleritis Allergies Contact lens wearing with irritation

ACUTE GENERAL Rx • • • • • •

CHRONIC Rx • • • •

WORKUP • • • • • • • • •

Intraocular pressure Gonioscopy Slit lamp examination Visual field examination GDx examination (laser scan of nerve fiber layer) Optic nerve evaluation Anterior chamber depth Cataract evaluation High hyperopia

Iridotomy Trabeculectomy Filter valves Other laser procedures

DISPOSITION Refer to ophthalmologist immediately.

REFERRAL This is an emergency—refer immediately to an ophthalmologist.

PEARLS & CONSIDERATIONS

LABORATORY TESTS • Blood sugar and CBC (if diabetes or inflammatory disease is suspected) • Visual field • GDx nerve fiber analysis

A

IV mannitol Pilocarpine -Blockers Diamox Laser iridotomy Anterior chamber paracentesis (as emergency treatment)

COMMENTS

B

• Do not use antihistamines or vasodilators with narrow-angle glaucoma. • After iridotomy, the majority of patients will be totally cured and will need no further medication and have no visual loss. • Lower socioeconomic status and higher levels of social deprivation are risk factors for delayed detection and probable worse outcomes in glaucoma.

SUGGESTED READINGS

FIGURE 1-20 Acute angle-closure glaucoma. A, Acutely elevated pressure produces an inflamed eye with corneal edema (note fragmented light reflex) and a middilated pupil. B, Slit lamp examination shows a very shallow central anterior chamber (space between cornea and iris) and no peripheral chamber. (From Palay D [ed]: Ophthalmology for the primary care physician, St Louis, 1997, Mosby.)

Foster PJ et al: Defining “occludable” angles in population surveys: drainage angle width, peripheral anterior synechiae, and glaucomatous optic neuropathy in East Asian people, Br J Ophthalmol 88(4):486, 2004. Fraser S et al: Deprivation and late presentation of glaucoma: case control study, BMJ 322:638, 2001. Gazzard G et al: Intraocular pressure and visual field loss in primary angle closure and primary open angle glaucomas, Br J Ophthalmol 87(6):720, 2003. Kapur SB: The lens and angle-closure glaucoma, J Cataract Refract Surg 27(2):176, 2001. Lam DS et al: Angle-closure glaucoma, Ophthalmology 109:1, 2002. AUTHOR: MELVYN KOBY, M.D.

DISEASES AND DISORDERS

Primary closed-angle glaucoma occurs when elevated intraocular pressure is associated with closure of the filtration angle or obstruction in the circulating pathway of the aqueous humor.

IMAGING STUDIES Section I

DEFINITION

• Old trauma • Chronic eye infections

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Gout

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BASIC INFORMATION DEFINITION Gout is a clinical disorder in which crystals of monosodium urate become deposited in tissue as a result of hyperuricemia. Gout and hyperuricemia can be classified as either primary or secondary if resulting from another disorder. ICD-9CM CODES 274.9 Gout

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 3 cases/1000 persons PREDOMINANT SEX: 95% males, rare in females before menopause

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Usually, initial attack in a single joint or an area of tenosynovium • Mainly a disease of the lower extremities • First site of involvement: classically, MP joint of the great toe • Another common site of acute attack: extensor tenosynovium on the dorsum of the midfoot • Severe pain and inflammation, which may be precipitated by exercise, dietary indiscretions, and physical or emotional stress • Attacks following illness or surgery • Presence of swelling, heat, redness, and other signs of inflammation (the physical findings simulating cellulitis) • Exquisite soft tissue tenderness • Fever, tachycardia, and other constitutional symptoms • Eventually, deposits of urate crystals (tophi) in the subcutaneous tissue

ETIOLOGY • Hyperuricemia and gout develop from excessive uric acid production, a decrease in the renal excretion of uric acid, or both. • Primary gout results from an inborn error of metabolism and may be attributed to several biochemical defects. • Secondary hyperuricemia may develop as a complication of acquired disorders (e.g., leukemia) or as a result of the use of certain drugs (e.g., diuretics).

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • •

Pseudogout Rheumatoid arthritis Osteoarthritis Cellulitis Infectious arthritis

Section II describes the differential diagnosis of acute monoarticular and oligoarticular arthritis.

WORKUP Hyperuricemia accompanying a typical history of monoarticular acute arthritis is usually sufficient to establish the diagnosis.

LABORATORY TESTS • • • •

Mild leukocytosis Elevated ESR Hyperuricemia Synovial aspirate: usually cloudy and markedly inflammatory in nature; urate crystals in fluid: needle-shaped and birefringent under polarized light

IMAGING STUDIES • Plain radiography to rule out other disorders • No typical findings in early gouty arthritis but late disease possibly associated with characteristic punched-out lesions and joint destruction

TREATMENT NONPHARMACOLOGIC THERAPY • Modification of diet (avoidance of foods high in purines [e.g., anchovies, organ meat, liver, spinach, mushrooms, asparagus, oatmeal, cocoa, sweetbreads]) and lifestyle • Treatment for obesity • Moderation in alcohol intake, no more than two drinks per day • Hypertension and its management requiring careful assessment and possibly nondiuretic drugs

ACUTE GENERAL Rx • Quick-acting NSAIDs such as ibuprofen • Colchicine (given PO or IV) • Corticosteroids or ACTH for those who are intolerant of NSAIDs or colchicine • Intraarticular cortisone when oral medication cannot be given • General measures, such as rest, elevation, and analgesics as needed until acute pain subsides. • Table 1-6 describes treatment options for gout

CHRONIC Rx • Prevention is achieved through normalization of serum urate concentration. • Uricosuric agents (e.g., probenecid) or xanthine oxidase inhibitors (allopurinol) are used in patients with recurrent attacks despite adequate dietary restrictions.

• A 24-hr urine collection is useful in deciding which antihyperuricemic agent is indicated. Allopurinol is generally used if the uric acid output is 900 mg/day on a regular diet. However, hyperuricemic therapy should not be started for at least 2 wk after the acute attack has resolved because it may prolong the acute attack and it can also precipitate new attacks by rapidly lowering the serum uric acid level. • Urinary uric acid hypoexcretors (700 mg/day) can be given probenecid (250 mg bid for 1 wk, then increased to 500 mg bid) to block absorption of uric acid. Probenecid should be started only after the acute attack of gout has completely subsided. • Colchicine 0.6 mg bid is indicated for acute gout prophylaxis before starting hyperuricemic therapy. It is generally discontinued 6 to 8 wk after normalization of serum urate levels. Longterm colchicine therapy (0.6 mg qd or bid) may be necessary in patients with frequent gout attacks despite the use of uricosuric agents. • Surgery usually limited to excision of large tophi and, occasionally, arthroplasty.

DISPOSITION • Musculoskeletal complications are usually limited to joint disease. • Surgical intervention may occasionally be indicated. • Renal disease is the most frequent complication of gout after arthritis; most gouty patients develop renal disease as a result of parenchymal urate deposition but the involvement is only slowly progressive and often has no effect on life expectancy. • Incidence of urolithiasis is increased, with 80% of calculi being uric acid stones.

REFERRAL For orthopedic consultation when joint destruction has occurred

PEARLS & CONSIDERATIONS COMMENTS • No significant correlation between coronary artery disease and gout • No indication to treat asymptomatic hyperuricemia • Acute attacks of gout occasionally associated with normal levels of uric acid • The main indication for prophylaxis is recurrent attacks of gouty joint inflammation, 3 or more per year

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Gout SUGGESTED READINGS

Wallace KL et al: Increasing prevalence of gout and hyperuricemia over 10 years among older adults in a managed care population, J Rheumatol 31(8):1582, 2004. AUTHOR: LONNIE R. MERCIER, M.D.

Treatment of Gout

Acute gout

Interval gout

Long-term treatment

Therapeutic goal: Terminate acute inflammatory attack. NSAIDs (preferred): Indomethacin, 50 mg qid, or ibuprofen, 800 mg tid (or other NSAIDs in full doses) (lower dose in renal insufficiency; contraindicated with peptic ulcer disease). OR Colchicine, oral (used infrequently): 0.6-1.2 mg (1-2 tablets), then 0.6 mg (1 tablet) q1-2h until attack subsides or until nausea, diarrhea, or GI cramping develops. Maximum total dose, 4-6 mg. If ineffective in 48 hr, do not repeat. Colchicine, IV (only if oral medication is precluded): 1-2 mg in 20 ml 0.9% saline infused slowly (extravasation causes tissue necrosis); dose may be repeated once in 6 hr. Few GI symptoms with IV use. Maximum total dose, 4 mg per attack. Monitor blood counts Steroids (if NSAIDs or colchicines are contraindicated or if oral medication is precluded, e.g., postoperatively): Triamcinolone acetonide, 60 mg IM, or ACTH, 40 U IM or 25 U by slow IV infusion, or prednisone, 20-40 mg daily. Intra-articular steroids may be used to treat a single inflamed joint: triamcinolone hexacetonide, 5-20 mg, or dexamethasone phosphate, 1-6 mg. Hypouricemic agents: Of no benefit for inflammatory attack and may initiate recurrent attack. Should not be started until attack has resolved, but ongoing use should not be interrupted during an attack.

Therapeutic goal: Prevent recurrent attacks.

Therapeutic goals: Prevent attacks, resolve tophi, maintain serum urate at 6 mg/dl. Colchicine, oral: 0.6-1.2 mg daily for 1-2 wk before initiating hypouricemic therapy and for several months afterward to prevent recurrent attacks during initial period of hypouricemic therapy. Allopurinol: Dose variable; usually 300 mg once daily, but up to 900 mg may be needed in occasional patient; dose should be reduced to 100 mg daily or every other day in patients with renal insufficiency. OR Uricosuric agent (reduced efficacy if creatinine clearance 80 ml; ineffective if 30 ml): Probenecid, 0.5-1 g bid, or sulfinpyrazone, 100 mg tid or qid; usually well tolerated, but may cause headache, GI upset, rash. Other: Diet—moderate protein, low fat; avoid excessive alcohol. Treat hypertension if present. For uric acid overexcretors or when initiating uricosuric agent: high fluid intake, particularly at night, to promote uric acid excretion in a dilute urine. Acetazolamide, 250 mg at bedtime, may be used to keep urine pH 6.

Colchicine, oral: 0.6-1.2 mg daily as prophylaxis against recurrent attacks.

Hypouricemic agent: Start only if indicated by frequent attacks, severe hyperuricemia, presence of tophi, urolithiasis, or urate overexcretion.

Other: Diet—moderate protein, low fat; avoid excessive alcohol. Treat hypertension if present. High fluid intake to promote uric acid excretion in a dilute urine (for uric acid overexcretors).

From Goldman L, Ausiello D (eds): Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders. ACTH, Adrenocorticotropic hormone; bid, twice daily; GI, gastrointestinal; IM, intramuscularly; IV, intravenously; NSAIDs, nonsteroidal antiinflammatory drugs; q1-2h, every 1 to 2 hours; qid, four times daily; tid, three times daily.

DISEASES AND DISORDERS

TABLE 1-6

Schlesinger N, Schumacher HR: Gout: can management be improved? Curr Opin Rheumatol 13:240, 2001. Terkeltaub RA: Gout, N Engl J Med 349:1647, 2003. Velilla-Moliner J et al: Podagra, is it always gout? Am J Emerg Med 22(4):320, 2004.

Section I

Agudelo CA, Wise CM: Gout: diagnosis, pathogenesis, and clinical manifestations, Curr Opin Rheumatol 13:234, 2001. Riedel AA et al: Compliance with allopurinol therapy among managed care enrollees with gout: a retrospective analysis of administrative claims, J Rheumatol 31(8):1575, 2004.

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Guillain-Barré Syndrome BASIC INFORMATION DEFINITION Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy (affects nerve roots and peripheral nerves), with predominant motor involvement. Maximal clinical weakness occurs within 4 wk of disease onset.

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SYNONYMS

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Acute polyneuropathy Ascending paralysis Postinfectious polyneuritis

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ICD-9CM CODES 357.0 Guillain-Barré

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EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: 0.6-1.9 cases/100,000 persons annually without geographical variation. Incidence increases with age. A slight male preponderance (1.25:1) also exists. PREDISPOSING FACTORS: Viral (HIV, CMV, EBV, influenza) and bacterial (Campylobacter jejuni, Mycoplasma pneumonia) infections; systemic illness (Hodgkin’s lymphoma, immunizations)

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Symmetric weakness, initially involving proximal muscles, subsequently involving both proximal and distal muscles; difficulty in ambulating, getting up from a chair, or climbing stairs • Depressed or absent reflexes bilaterally • Minimal to moderate glove and stocking paresthesias/dysesthesia/anesthesia and/or back pain • Pain (caused by involvement of posterior nerve roots) may be prominent • Autonomic abnormalities (brady- or tachyarrhythmias, hypo- or hypertension) • Respiratory insufficiency (caused by weakness of bulbar/intercostal muscles) • Facial paresis, ophthalmoparesis, dysphagia (secondary to cranial nerve involvement)

ETIOLOGY Unknown. Preceding infectious illness 14 wk before disease onset in 66% of patients. Humoral and cell-mediated immune attack of peripheral nerve myelin, Schwann cells; sometimes with axonal involvement

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Toxic peripheral neuropathies: heavy metal poisoning (lead, thallium, arsenic), medications (vincristine, disul-

•

firam), organophosphate poisoning, hexacarbon (glue sniffer’s neuropathy) Nontoxic peripheral neuropathies: acute intermittent porphyria, vasculitic polyneuropathy, infectious (poliomyelitis, diphtheria, Lyme disease); tick paralysis Neuromuscular junction disorders: myasthenia gravis, botulism, snake envenomations Myopathies; such as polymyositis, acute necrotizing myopathies caused by drugs Metabolic derangements such as hypermagnesemia, hypokalemia, hypophosphatemia, thyrotoxicosis Acute central nervous system disorders such as basilar artery thrombosis with brainstem infarction, brainstem encephalomyelitis, transverse myelitis, or spinal cord compression Hysterical paralysis or malingering

WORKUP 1. Exclude other causes based on clinical history, examination, and laboratory tests. 2. Lumbar puncture (may be normal in the first 1-2 wk of the illness) • Typical findings include elevated CSF protein with few mononuclear leukocytes (albuminocytologic dissociation) in 80%-90% of patients. Elevated CSF cell counts is an expected feature in cases associated with HIV seroconversion. 3. EMG/NCS: May be normal in the first 10-14 days of the disease. The earliest electrodiagnostic abnormality is prolongation or absence of H-reflexes.

LABORATORY TESTS • CBC may reveal early leukocytosis with left shift. Electrolytes to exclude metabolic causes • Heavy metal testing, urine porphyria screen, creatine kinase, HIV titers, neuroimaging of the brain and spinal cord if diagnosis uncertain

TREATMENT NONPHARMACOLOGIC THERAPY • Close monitoring of respiratory function (frequent measurements of vital capacity, negative inspiratory force and pulmonary toilet), because respiratory failure is the major complication in GBS • Frequent repositioning of patient to minimize formation of pressure sores • Prevention of thromboembolism with antithrombotic stockings and SC heparin (5000 U q12h) in nonambulatory patients • Emotional support and social counseling

ACUTE GENERAL Rx • Infusion of IV immunoglobulins (IVIg; 0.4 g/kg/day for 5 days). Always check serum IgA levels before infusion to prevent anaphylaxis in deficient patients. • Early therapeutic plasma exchange (TPE or plasmapheresis: 200-250 ml/ kg over 5 sessions qod), started within 7 days of onset of symptoms, is beneficial in preventing paralytic complications in patients with rapidly progressive disease. It is contraindicated in patients with cardiovascular disease (recent MI, unstable angina), active sepsis, and autonomic dysfunction. • Mechanical ventilation may be needed if FVC is 12 to 15 ml/kg, vital capacity is rapidly decreasing or is 1000 ml, negative inspiratory force 20 cm H2rO, PaO2 is 70, the patient is having significant difficulty clearing secretions or is aspirating.

CHRONIC Rx • Ventilatory support: may be necessary in 10% to 20% of patients. Adequate fluid/electrolyte support and nutrition necessary, especially in patients with dysautonomia or bulbar dysfunction • Aggressive nursing care to prevent decubiti, infections, fecal impactions, and pressure nerve palsies • Monitoring and treatment of autonomic dysfunction (bradyarrhythmias or tachyarrhythmias, orthostatic hypotension, systemic hypertension, altered sweating) • Treatment of back pain and dysesthesia with low-dose tricyclics, gabapentin, etc. • Stress ulcer prevention in patients receiving ventilator support • Physical and occupational therapy rehabilitation, including supportive devices

DISPOSITION • Mortality is approximately 5%-10%. A recent study showed 62% complete recovery, 14% mild weakness, 9% moderate weakness, 4% bed-bound or ventilated, and 8% dead at 1 yr. • Predictors for poor recovery (inability to walk independently at 1 yr): older age, preceding diarrheal illness, recent CMV infection, fulminant or rapidly progressing course, ventilatory dependence, reduced motor amplitudes (20% normal), or inexcitable nerves on NCS.

REFERRAL Tracheostomy may be necessary in patients with prolonged ventilatory support. Percutaneous endoscopic gastrostomy may also be required.

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COMMENTS

Gorson KC, Ropper AH: Guillain-Barré syndrome (acute inflammatory demyelinating neuropathy) and related disorders. In: Katirji B et al. (eds): Neuromuscular disorders in clinical practice. Boston, 2002, Butterworth-Heinemann.

Kuwabara S: Guillain-Barré syndrome: epidemiology, pathophysiology and management, Drugs 64:597, 2004. AUTHOR: EROBOGHENE E. UBOGU, M.D.

DISEASES AND DISORDERS

Patient education information may be obtained from the Guillain-Barré Foundation International, Box 262, Wynnewood, PA 19096; phone: (610) 667-0131.

SUGGESTED READINGS

Section I

PEARLS & CONSIDERATIONS

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Heart Failure, Congestive BASIC INFORMATION DEFINITION Congestive heart failure (CHF) is a pathophysiologic state characterized by congestion in the pulmonary or systemic circulation. It is caused by the heart’s inability to pump sufficient oxygenated blood to meet the metabolic needs of the tissues. The various classifications for heart failure are described as follows. The American College of Cardiology and the American Heart Association describe the following four stages of heart failure: A. At high risk for heart failure, but without structural heart disease or symptoms of heart failure (e.g., CAD, hypertension) B. Structural heart disease but without symptoms of heart failure C. Structural heart disease with prior or current symptoms of heart failure D. Refractory heart failure requiring specialized interventions The New York Heart Association (NYHA) defines the following functional classes: I. Asymptomatic II. Symptomatic with minimal exertion III. Symptomatic with moderate exertion IV. Symptomatic at rest

SYNONYMS CHF Cardiac failure Heart failure ICD-9CM CODES 428.0 Congestive heart failure

EPIDEMIOLOGY & DEMOGRAPHICS • CHF is the most common admission diagnosis (20%) in elderly patients. • Heart failure occurs in 4.7 million persons in the U.S. and is the discharge diagnosis in 3.5 million hospitalizations annually.

PHYSICAL FINDINGS & CLINICAL PRESENTATION The findings on physical examination in patients with CHF vary depending on the severity and whether the failure is rightsided or left-sided. • Common clinical manifestations are: 1. Dyspnea on exertion initially, then with progressively less strenuous activity, and eventually manifesting when patient is at rest; caused by increasing pulmonary congestion 2. Orthopnea caused by increased venous return in the recumbent position

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3. Paroxysmal nocturnal dyspnea (PND) resulting from multiple factors (increased venous return in the recumbent position, decreased Pao2, decreased adrenergic stimulation of myocardial function) 4. Fatigue, reduced exercise tolerance, lethargy resulting from low cardiac output, deterioration in ADLs 5. Cognitive impairment, new, or worsening of underlying dementia (i.e., delirium) Patients with failure of the left side of the heart may have the following abnormalities on physical examination: pulmonary rales, tachypnea, S3 gallop, cardiac murmurs (AS, AR, MR), paradoxic splitting of S2. Patients with failure of right side of the heart manifest with jugular venous distention, peripheral edema, perioral and peripheral cyanosis, congestive hepatomegaly, ascites, hepatojugular reflux. In patients with heart failure, elevated jugular venous pressure and a third heart sound each are independently associated with adverse outcomes. Acute precipitants of CHF exacerbations are: noncompliance with salt restriction, pulmonary infections, arrhythmias, medications (e.g., calcium channel blockers/antiarrhythmic agents), and failed attempts at reductions in CHF therapy.

ETIOLOGY LEFT VENTRICULAR FAILURE • Systemic hypertension • Valvular heart disease (AS, AR, MR) • Cardiomyopathy, myocarditis • Bacterial endocarditis • Myocardial infarction • IHSS Left ventricular failure is further differentiated according to systolic dysfunction (low ejection fraction) and diastolic dysfunction (normal or high ejection fraction), or “stiff ventricle.” It is important to make this distinction because treatment is different (see Treatment). Patients with heart failure and a normal ejection fraction have abnormalities in active relaxation and passive stiffness (see Heart Failure, Diastolic in Section I). • Common causes of systolic dysfunction are post-MI, cardiomyopathy, myocarditis. • Causes of diastolic dysfunction are hypertensive cardiovascular disease, restrictive cardiomyopathy, ischemic heart disease (see Heart Failure, Diastolic in Section I). RIGHT VENTRICULAR FAILURE • Valvular heart disease (mitral stenosis) • Pulmonary hypertension • Bacterial endocarditis (right-sided) • Right ventricular infarction

BIVENTRICULAR FAILURE • Left ventricular failure • Cardiomyopathy • Myocarditis • Arrhythmias • Anemia • Thyrotoxicosis • AV fistula • Paget’s disease • Beriberi

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • •

Cirrhosis Nephrotic syndrome Venous occlusive disease COPD, asthma Pulmonary embolism ARDS Heroin overdose Pneumonia

WORKUP • Standard 12-lead ECG is useful to diagnose ischemic heart disease and obtain information about rhythm abnormalities.

LABORATORY TESTS • CBC (to rule out anemia, infections), BUN, creatinine, liver enzymes, TSH • B-type natriuretic peptide is a cardiac neurohormone specifically secreted from the ventricles in response to volume expansion and pressure overload. Elevated levels are indicative of CHF. Bedside measurement of B-type natriuretic peptide is useful in establishing or excluding the diagnosis of CHF in patients with acute dyspnea.

IMAGING STUDIES • Chest x-ray examination: 1. Pulmonary venous congestion 2. Cardiomegaly with dilation of the involved heart chamber 3. Pleural effusions • Two-dimensional echocardiography is critical to assess global and regional left ventricular function and estimate ejection fraction. The current standard of care requires echocardiography as part of the management of CHF. • Exercise stress testing may be useful for evaluating concomitant coronary disease and assessing degree of disability in selected patients. • Cardiac catheterization is an excellent method to evaluate ventricular diastolic properties, significant coronary artery disease, or valvular heart disease; however, it is invasive. The decision to perform cardiac catheterization should be individualized.

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Heart Failure, Congestive TREATMENT

PHARMACOLOGIC THERAPY TREATMENT OF CHF SECONDARY TO SYSTOLIC DYSFUNCTION 1. Diuretics: indicated in patients with systolic dysfunction and volume overload. The most useful approach to selecting the dose of, and monitoring the response to, diuretic therapy is by measuring body weight, preferably on a daily basis. a. Furosemide: 20 to 80 mg/day produces prompt venodilation and diuresis. IV therapy may produce diuresis when oral therapy has failed; when changing from IV to oral furosemide, doubling the dose is usually necessary to achieve an equal effect. Monitor serum potassium every 6 months (every 3 months if digitalis is also used). b. Thiazides are not as powerful as furosemide but are useful in mild to moderate CHF. Monitor serum potassium every 12 months (every 6 months if digitalis is also used). Thiazides are not effective when creatinine clearance is 20 ml/min. c. The addition of metolazone to furosemide enhances diuresis. Monitor potassium frequently. d. Blockade of aldosterone receptors by spironolactone (12.5 to 25 mg qd) used in conjunction with ACE inhibitors reduces both mortality and morbidity in patients with severe CHF. It is generally not associated with hyperkalemia when used in low doses; however, serum electrolytes and renal function should be closely monitored after initiation of therapy and when changing doses. Spironolactone use should be considered in patients with recent or recurrent class IV (NYHA) symptoms.

DISEASES AND DISORDERS

• Identify and correct precipitating factors (i.e., anemia, thyrotoxicosis, infections, increased sodium load, -blockers, medical noncompliance). • Decrease cardiac workload (restrict patients’ activity) only during periods of acute decompensation. The risk of thromboembolism during this period can be minimized by using heparin 5000 U SC q12h in hospitalized patients. In patients with mild to moderate symptoms, aerobic training may improve symptoms and exercise capacity. • Restrict sodium intake to 3 g/day. • Restricting fluid intake to 2 L or less may be useful in patients with hyponatremia.

4. -blockers: All patients with stable NYHA class II or III heart failure caused by left ventricular systolic dysfunction should receive a blocker unless they have a contraindication to its use or are intolerant to it. -blockers are especially useful in patients who remain symptomatic despite therapy with ACE inhibitors and diuretics. Effective agents are carvedilol (Coreg) 3.125 mg bid, bisoprolol 1.25 mg qd, or metoprolol 12.5 mg bid initially, titrated upward as tolerated. 5. Digitalis is useful because of its positive inotropic and vagotonic effects in patients with CHF secondary to systolic dysfunction; it is of limited value in patients with mild CHF and normal sinus rhythm. It is more beneficial in patients with rapid atrial fibrillation, severe CHF, or ejection fraction of 30%; it can be added to diuretics and ACE inhibitors in patients with severe CHF. In patients with chronic heart failure and normal sinus rhythm, digoxin does not reduce mortality, but it does reduce the rate of hospitalization both overall and for worsening heart failure. Its beneficial effects are found with a low dose that results in a serum concentration of approximately 0.7 ng/ml. Higher doses may be detrimental. 6. Direct vasodilating drugs (nesiritide, hydralazine, isosorbide) are useful in the therapy of systolic dysfunction with CHF because they can reduce the systemic vascular resistance and pulmonary venous pressure, especially when used in combination. 7. Anticoagulants: a. Anticoagulation is not recommended for patients in sinus rhythm and no prior history of stroke, left ventricular thrombi, or arteriolar emboli. b. Anticoagulation therapy is appropriate for patients with heart failure and atrial fibrillation or a history of embolism. c. Aspirin is appropriate for all patients with ischemic heart disease. 8. Surgical revascularization should be considered in patients with both heart failure and severe limiting angina. 9. Atriobiventricular pacing significantly improves exercise tolerance and quality of life in patients with chronic heart failure and intraventricular conduction delay. 10. Obstructive sleep apnea has an adverse effect on heart failure. Recognition and treatment of coexisting obstructive sleep apnea by continuous positive airway pressure reduces systolic blood pressure and improves left ventricular systolic function.

Section I

NONPHARMACOLOGIC THERAPY

e. Elderly patients have a blunted thirst sensation. They also may have restricted access to water because of mobility impairment or cognitive deficits. As a result, intravascular volume depletion and prerenal failure is a common complication of diuretic therapy. Patients with mild baseline renal failure (e.g., diabetic nephropathy) and patients dependent on others for fluid intake (e.g., nursing home patients) are particularly at risk and need a close monitoring (for clinical signs of dehydration and laboratory evidence of prerenal failure: high BUN/creatinine ratio). f. Monitoring fluid intake and output (I&O) is important and appropriate in the hospital. (If a urinary catheter is used for this purpose, its duration should be minimized.) In the nursing home setting, I&O is often unreliable and in outpatients it is impractical. Daily to weekly weight should be monitored in such situations instead of I&O. For reliable patients, a weight-based diuretic sliding scale can be recommended. 2. ACE inhibitors: a. Cause dilation of the arteriolar resistance vessels and venous capacity vessels, thereby reducing both preload and afterload. b. Are associated with decreased mortality and improved clinical status. c. Can be used as first line therapy or can be added to diuretics. d. Therapy with ACE inhibitors should be initiated at low dose (e.g., captopril 6.25 mg tid or enalapril 2.5 mg bid) to prevent hypotension and titrated up to high doses if tolerated. e. Contraindications to use of ACE inhibitors are renal insufficiency (creatinine 3.0 or creatinine clearance 30 ml/min), renal artery stenosis, persistent hyperkalemia (K 5.5 mEQ/L), symptomatic hypotension, and history of adverse reactions (e.g., angioedema). f. ACE inhibitors and -blockers can be used together. 3. Angiotensin II receptor blockers (ARBS) block the A-II type 1 (AT) receptor, which is responsible for many of the deleterious effects of angiotensin II. These receptors are potent vasoconstrictors that may contribute to the impairment of LV function. They are useful in patients unable to tolerate ACE inhibitors because of angioedema or intractable cough. They can also be used in combination with a -blocker.

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Heart Failure, Congestive TREATMENT OF CHF SECONDARY TO STRUCTURAL CARDIAC ABNORMALITIES 1. Aortic stenosis a. Diuretics. b. Contraindicated medications: ACE inhibitors, ARBs, nitrates, digitalis (except to control rate of atrial fibrillation). c. Aortic valve replacement in patients with critical stenosis. 2. Aortic insufficiency and mitral regurgitation a. ACE inhibitors increase cardiac output and decrease pulmonary wedge pressure. They are agents of choice along with diuretics. b. Hydralazine combined with nitrates can be used if ACE inhibitors are not tolerated. c. Surgery. 3. IHSS a. -blockers or verapamil. b. Contraindicated medications (they increase outlet obstruction by decreasing the size of the left ventricle in end systole): diuretics, digitalis, ACE inhibitors, ARBs, hydralazine. c. Restoration of intravascular volume with IV saline solution if necessary in acute pulmonary edema. d. Septal myotomy, DDD pacing are useful in selected patients. 4. Mitral stenosis a. Diuretics. b. Control of the heart rate and atrial fibrillation with digitalis, verapamil, and/or -blockers is critical to allow emptying of left atrium and relief of pulmonary congestion.

c. Repairing or replacing the mitral valve is indicated if CHF is not readily controlled by the previously discussed measures. d. Balloon valvuloplasty is useful in selected patients. TREATMENT OF DIASTOLIC HEART FAILURE See Heart Failure, Diastolic in Section I.

DISPOSITION • Annual mortality ranges from 10% in stable patients with mild symptoms to 50% in symptomatic patients with advanced disease. • Sudden death secondary to ventricular arrhythmias occurs in 40% of patients with heart failure. • Implantable cardiac defibrillator device in selected patients. • In patients with advanced heart failure and a prolonged QRS interval, cardiacresynchronization therapy decreases the combined risk of death from any cause or first hospitalization and, when combined with an implantable defibrillator, significantly reduces mortality. • Intensive home care by nurses with expertise in the management of heart failure can improve symptom control and reduce hospitalization. • Cardiac transplantation has a 5-yr survival rate of 70% in many centers and represents a viable option in selected patients. • The use of a left ventricular assist device in patients with advanced heart failure can result in a clinically meaningful survival benefit and improve quality of life.

SUGGESTED READINGS Aurigemma GP, Gaasch WH: Diastolic heart failure, N Engl J Med 351:1097-1105, 2004. Bristow MR et al: Cardiac-resynchronization therapy with or without an implantable defibrillator in advanced chronic heart failure, N Engl J Med 350:2140-2150, 2004. Goldstein S: Benefits of -blocker therapy in heart failure, Arch Intern Med 162:641, 2002. Gutierrez C, Blanchard DG: Diastolic heart failure: challenges of diagnosis and treatment, Am Fam Physician 69:2609-2616, 2004. Jessup M, Brozena S: Heart failure, N Engl J Med 348:2007, 2003. King DE et al: Acute management of heart failure, Am Fam Physician 66:249, 2002. Kukin ML: -blockers in chronic heart failure, Mayo Clin Proc 77:1199, 2002. Maisel AS et al: Rapid measurement of B-type natriuretic peptide in the emergency diagnosis of heart failure, N Engl J Med 347:161, 2002. Mueller C et al: Use of B-type natriuretic peptide in the evaluation and management of acute dyspnea, N Engl J Med 350:647-654, 2004. Nohria A et al: Medical management of advanced heart failure, JAMA 287:628, 2002. Pfeffer MA et al: Valsartan, captopril, or both in myocardial infarction complicated by heart failure, left ventricular dysfunction, or both, N Engl J Med 349(20):1893-1906, 2003. Zile MR et al: Diastolic heart failure—abnormalities in active relaxation and passive stiffness of the left ventricle, N Engl J Med 350:1953-1959, 2004. AUTHORS: FRED F. FERRI, M.D., and TOM J. WACHTEL, M.D.

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Heart Failure, Diastolic BASIC INFORMATION

SYNONYMS Congestive heart failure with preserved LV function ICD-9CM CODES 428.0 Congestive heart failure (includes all causes of heart failure)

EPIDEMIOLOGY & DEMOGRAPHICS • 10% to 15% progression over 5 yr from diastolic dysfunction to diastolic heart failure • One third patients with CHF • Ratio increases with age; highest in 75 years of age • Mortality rate about half that of systolic heart failure • Morbidity (e.g., hospitalization) same for diastolic and systolic heart failure • Incidence/prevalence are unknown

PATHOPHYSIOLOGY • Patients with diastolic heart failure have low stroke volume despite normal ejection fraction. • Passive stiffness caused by:  Increased myocardial mass (hypertensive patient)  Alteration in collagen (CAD patient) • Impaired active relaxation:  When chamber compliance is reduced, a small increase in blood volume causes a large increase in left atrial pressure. • In addition, tachycardia, which may be somewhat helpful to patients with systolic dysfunction, is deleterious when the cause of heart failure is diastolic dysfunction. Indeed, a “stiff” ventricle needs more time to fill during diastole. Thus tachycardia is similar in effect to a pump that loses its prime.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS

Uncontrolled hypertension Atrial fibrillation Other causes of tachycardia Myocardial ischemia Medication noncompliance Dietary noncompliance (salt) Anemia Renal failure Nonsteroidal antiinflammatory drugs (NSAIDs) including COX 2s • Thioglitazones (TZDs)

• Pulmonary pathology  COPD  Asthma  Pneumonia  ARDS  Pulmonary embolism • Volume overload  Cirrhosis  Portal hypertension  Nephrotic syndrome  Lower extremity venous insufficiency • Pericardial effusion with tamponade • Constrictive pericarditis • Other causes of CHF (see Heart Failure, Congestive in Section I)

PHYSICAL FINDINGS

WORKUP

The symptoms and signs of systolic and diastolic heart failure are indistinguishable (see Heart Failure, Congestive in Section I). Symptoms include: • Dyspnea on exertion • Orthopnea • Paroxysmal nocturnal dyspnea • Reduced exercise capacity, fatigue Physical findings: • Tachycardia • Hypertension • Hypotension • Pulmonary crackles in the lower parts of the lungs

• Laboratory  CBC  BUN and creatinine  ALT  Alkaline, phosphatase  TSH  Cardiac enzymes  B-type natriuretic peptide (BNP) • ECG • Chest x-ray • Two-dimensional echocardiography • It is probably inappropriate in the 21st century to manage CHF without having the results of a reasonably recent echo.

PRECIPITANTS OF DIASTOLIC HEART FAILURE • • • • • • • • •

TREATMENT • Pulmonary edema associated with diastolic heart failure  Diuretics  Oxygen  Morphine  Nitrates 1. Treat ischemia if present. 2. Treat severe hypertension (“hypertensive crisis”) if present. 3. Treat tachycardia (may require urgent cardioversion of atrial fibrillation). • Chronic management for patients with diastolic heart failure  Reduce the congestive state. 1. Salt restriction 2. Diuretics 3. ACE inhibitors 4. Angiotensin II—receptor blockers  Maintain atrial contraction and prevent tachycardia. 1. -blockers 2. Calcium channel blockers 3. Cardioversion of atrial fibrillation 4. Sequential atrioventricular pacing 5. Radiofrequency ablation modification of atrioventricular node and pacing  Treat and prevent myocardial ischemia. 1. Nitrates 2. -blockers 3. Calcium channel blockers 4. Coronary-artery bypass surgery, percutaneous coronary intervention  Control hypertension. 1. Antihypertensive agents

SUGGESTED READINGS Aurigemma GP, Gaasch WH: Diastolic heart failure, N Engl J Med 351:1097-1105, 2004. Redfield MM: Understanding “diastolic” heart failure, N Engl J Med 350:1930-1931, 2004. Vasan RS: Diastolic heart failure, BMJ USA 4:80-84, 2004. Zile MR, Baicu CF, Gaasch WH: Diastolic heart failure: abnormalities in active relaxation and passive stiffness of the left ventricle, N Engl J Med 350:1953-1959, 2004. AUTHOR: TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

DIASTOLIC DYSFUNCTION Abnormality of diastolic distensibility, filling, or relaxation of the left ventricle (LV) DIASTOLIC HEART FAILURE Patient with previous pathophysiology and a normal LV ejection fraction who develops congestive heart failure (CHF) symptoms

• Definite diastolic heart failure: LVEF 50%; “probable” diastolic heart failure: LVEF 40%-50%. • Diagnosis of diastolic heart failure assumes that other diagnoses (e.g., mitral regurgitation, aortic regurgitation, constrictive pericarditis) have been excluded. • Doppler echo and cardiac cath can also help.

Section I

DEFINITION

• Signs of pleural effusion, more likely right-sided: dullness to percussion, reduced breath sounds • Heart murmurs, S3 gallop • Peripheral edema • Hepatomegaly • Hepatojugular reflux • Acrocyanosis

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Heat Exhaustion and Heat Stroke BASIC INFORMATION DEFINITION HEAT EXHAUSTION: An illness resulting from prolonged heavy activity in a hot environment with subsequent dehydration, electrolyte depletion, and rectal temperature 37.8° C but 40° C. HEAT STROKE: A life-threatening heat illness characterized by extreme hyperthermia, dehydration, and neurologic manifestations (core temperature 40° C).

Epilepsy Thyroid storm Acute cocaine intoxication Malignant hyperthermia Heat exhaustion can be differentiated from heat stroke by the following: 1. Essentially intact mental function and lack of significant fever in heat exhaustion 2. Mild or absent increases in CPK, AST, LDH, ALT in heat exhaustion

WORKUP • Heat stroke: comprehensive history, physical examination, and laboratory evaluation • Heat exhaustion: in most cases, laboratory tests are not necessary for diagnosis

SYNONYMS Heat illness Hyperthermia ICD-9CM CODES 992.0 Heat stroke 992.5 Heat exhaustion

LABORATORY TESTS

EPIDEMIOLOGY & DEMOGRAPHICS • Heat exhaustion and stroke occur more frequently in elderly patients, especially those taking diuretics or medications that impair heat dissipation (e.g., phenothiazines, anticholinergics, antihistamines, -blockers).

PHYSICAL FINDINGS & CLINICAL PRESENTATION HEAT EXHAUSTION: • Generalized malaise, weakness, headache, muscle and abdominal cramps, nausea, vomiting, hypotension, and tachycardia • Rectal temperature is usually normal • Sweating is usually present HEAT STROKE: • Neurologic manifestations (seizures, tremor, hemiplegia, coma, psychosis, and other bizarre behavior) • Evidence of dehydration (poor skin turgor, sunken eyeballs) • Tachycardia, hyperventilation • Skin is hot, red, and flushed • Sweating is often (not always) absent, particularly in elderly patients

ETIOLOGY • Exogenous heat gain (increased ambient temperature) • Increased heat production (exercise, infection, hyperthyroidism, drugs) • Impaired heat dissipation (high humidity, heavy clothing, elderly patients, drugs [phenothiazines, anticholinergics, antihistamines, butyrophenones, amphetamines, cocaine, alcohol, blockers])

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Infections (meningitis, sepsis) • Head trauma

• • • • •

encephalitis,

Laboratory abnormalities may include the following: • Elevated BUN, creatinine, Hct • Hyponatremia or hypernatremia, hyperkalemia or hypokalemia • Elevated LDH, AST, ALT, CPK, bilirubin • Lactic acidosis, respiratory alkalosis (secondary to hyperventilation) • Myoglobinuria, hypofibrinogenemia, fibrinolysis, hypocalcemia

TREATMENT • Treatment of heat exhaustion consists primarily of placing the patient in a cool, shaded area and providing rapid hydration and salt replacement. 1. Fluid intake should be at least 2 L q4h in patients without history of CHF. 2. Salt replacement can be accomplished by using one-quarter teaspoon of salt or two 10-grain salt tablets dissolved in 1 L of water. 3. If IV fluid replacement is necessary in elderly patients, consider using D51⁄2NS IV with rate titrated to cardiovascular status. • Patients with heat stroke should undergo rapid cooling. 1. Remove the patient’s clothes and place the patient in a cool and well-ventilated room. 2. If unconscious, position patient on his or her side and clear the airway. Protect airway and augment oxygenation (e.g., nasal O2 at 4 L/min to keep oxygen saturation 90%). 3. Monitor body temperature every 5 min. Measurement of the patient’s core temperature with a rectal probe is recommended. The goal is to reduce the body temperature to 39° C (102.2° F) in 30 to 60 min. 4. Spray the patient with a cool mist and use fans to enhance airflow

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11.

12.

over the body (rapid evaporation method). Immersion of the patient in ice water, stomach lavage with iced saline solution, intravenous administration of cooled fluids, and inhalation of cold air are advisable only when the means for rapid evaporation are not available. Immersion in tepid water (15° C [59° F]) is preferred over ice water immersion to minimize risk of shivering. Use of ice packs on axillae, neck, and groin is controversial because they increase peripheral vasoconstriction and may induce shivering. Antipyretics are ineffective because the hypothalamic set point during heat stroke is normal despite the increased body temperature. Intubate a comatose patient, insert a Foley catheter, and start nasal O2. Continuous ECG monitoring is recommended. Insert at least two large-bore IV lines and begin IV hydration with NS or Ringer’s lactate. Draw initial lab studies: electrolytes, CBC, BUN, creatinine, AST, ALT, CPK, LDH, glucose, INR, PTT, platelet count, Ca2, lactic acid, ABGs. Treat complications as follows: a. Hypotension: vigorous hydration with normal saline or Ringer’s lactate. b. Convulsions: diazepam 5 to 10 mg IV (slowly). c. Shivering: chlorpromazine 10 to 50 mg IV. d. Acidosis: use bicarbonate judiciously (only in severe acidosis). Observe for evidence of rhabdomyolysis, hepatic, renal, or cardiac failure and treat accordingly.

DISPOSITION Most patients recover completely within 48 hr. Mortality can exceed 30% in patients with prolonged and severe hyperthermia.

SUGGESTED READINGS Bouchama A, Knochel JP: Heat stroke, N Engl J Med 346:1978, 2002. Wexler RK: Evaluation and treatment of heatrelated illness, Am Fam Physician 65:230, 2002. AUTHOR: FRED F. FERRI, M.D.

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Hematuria BASIC INFORMATION

ICD-9CM CODES 599.7

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE (IN U.S.): Studies report a range from 0.18% to 16.1%. The prevalence is directly associated with age. In patients over 50 years of age, the prevalence is estimated at 13%. PREDOMINANT SEX: Several studies have reported a slightly higher prevalence in women. GENETICS: A family history of renal failure exists in patients with hereditary nephritis and thin basement membrane disease.

ETIOLOGY To identify the cause of hematuria, the physician should differentiate between a glomerular (medical) or nonglomerular (urologic) source of bleeding. The three most common causes of glomerular hematuria include IgA nephropathy (hematuria, hypertension, rising creatinine), thin basement membrane disease (hematuria, no extrarenal manifestations, rarely renal failure), and hereditary nephritis (hematuria, rising creatinine, 2 gm proteinuria, sensorineural deafness). Nonglomerular causes of hematuria can include lesions in either the kidney or upper urinary tract: neoplasm, nephrolithiasis, cystic disease, and papillary necrosis. Metabolic defects (hypercalciuria or hyperuricosuria) are associated with hematuria. Lower urinary tract lesions, chiefly infectious or neoplastic diseases of the bladder, urethra, and prostate can result in hematuria. Gross hematuria suggests a urologic cause of bleeding and is associated with a much higher risk for a urologic cancer. The suspicion of neoplasm increases with age, with no specific age threshold.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS False-positive tests can result from substances other than hemoglobin such as myoglobin, vitamin C, beets, and rhubarb. A urinalysis dipstick, which is positive for “blood” but not associated with RBC on microscopic urinalysis, is not hematuria.

LABORATORY TESTS Initially a repeat urinalysis should be obtained in a few days. If microscopic hematuria is absent on a repeat test, no further evaluation is warranted if the cause of hematuria is infectious unless the patient has risk factors for bladder cancer. If hematuria is present on repeat testing, a basic chemistry and microscopy are obtained from the patient. Combination of dysmorphic red cells and red cell casts suggests glomerular disease. An elevated creatinine suggests a renal source of the bleeding. A urine dipstick that is positive for protein should then be followed by the spot protein-tocreatinine ratio (P/Cr) or a 24-hour urine protein. A P/Cr greater than 0.3 or 300 mg in a 24-hour period suggests a glomerular source to the bleeding. Anatomic evaluation is indicated in all high-risk patients (age greater than 40 or risk factors for bladder cancer) with hematuria.

IMAGING STUDIES • Glomerular: no imaging studies will differentiate between the glomerular disease. Biopsy is indicated with worsening renal failure and progressive proteinuria. • Upper urinary tract: in patients suspected of stone disease, a computer tomography scan without contrast is appropriate. If there is no suspicion for stone disease, CT urography is obtained first without contrast followed by imaging with contrast dye. Ultrasound and excretory urography are most often followed by additional imaging and therefore are not considered cost-saving tests. If a CT scan cannot be performed (unavailable, contrast hypersensitivity, renal failure), ultrasound is the most reasonable alternative test. • Lower urinary tract: cystoscopy is indicated in all the following patients: over the age of 40 or under the age of 40 in a whom a source of hematuria is not identified with upper urinary tract imaging, with risk factors for bladder cancer, gross hematuria, a urine cytology showing neoplastic cells.

TREATMENT ACUTE GENERAL Rx Treat any infection with appropriate antibiotic therapy, nephrolithiasis with hydration and narcotic analgesia, hematuria resulting from metabolic defects will resolve with correction of offending electrolyte imbalance. A risk/benefit analysis including goals of care and quality-of-life expectations should be discussed with the patient before any surgical intervention for neoplastic disease.

CHRONIC Rx Management of hypertension and proteinuria should include ACE-inhibitor or angiotension receptor blocker therapy. Patients with neoplastic lesions in whom surgery is not performed and the hematuria results in a progressive anemia may require intermittent RBC transfusion therapy.

REFERRAL When microscopic hematuria accompanies proteinuria or renal insufficiency a nephrology consult is warranted. A nephrology referral should also be obtained if polycystic kidney disease is suspected.

PEARLS & CONSIDERATIONS COMMENTS Isolated glomerular hematuria requires repeat testing for proteinuria and renal insufficiency at 6 months and then annually if stable. Urology referral is required whenever cystoscopy will aid in evaluation of hematuria. Isolated urologic hematuria can be monitored every 3 months for 1 yr, yearly for 3 yr, and episodically thereafter. • The U.S. Preventative Task Force does not recommend routine screening for hematuria due to lack of evidence for effectiveness. • A diagnosis of nephrolithiasis does not rule out the possibility of an associated urologic malignancy. • A finding of hematuria in a patient with a recent urinary catheterization should be discarded and have a repeat urinalysis done in 2 to 3 weeks.

SUGGESTED READINGS Brehmer, M: Imaging for microscopic hematuria, Curr Opin Urol 12:155-159, 2002. Cohen RA, Brown RS: Microscopic hematuria, N Engl J Med 348:2330-2338, 2003. Jaffe JS et al: A new diagnostic algorithm for the evaluation of microscopic hematuria, Urology 57:889-894, 2001. Khadra M, Pickard R, Charlton M: A prospective analysis of 1930 patients with hematuria to evaluate current diagnostic practice., J Urol 163:524-527, 2000. AUTHOR: MICHAEL P. GERARDO, D.O., M.P.H.

DISEASES AND DISORDERS

Hematuria is classified as either microscopic or gross in nature. In microscopic hematuria, two or more red blood cells per high power field are identified in a centrifuged urine specimen. In gross hematuria, the blood is visualized by the naked eye. If the RBC per high power field is greater than 20, a tint to the urine will be visible on urinalysis.

Risk factors for bladder cancer: 65 years of age, cigarette smoking, occupational exposure to chemicals (leather, dye, rubber or tire manufacturing), heavy phenacetin use, previous cyclophosphamide treatment, aristolochic acid (found in some weight loss medications). Many clinicians recommend that patients who develop hematuria during anticoagulation therapy should not only have the anticoagulation indices monitored but also undergo an evaluation of the cause of hematuria.

Section I

DEFINITION

WORKUP

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Hemorrhoids BASIC INFORMATION DEFINITION A hemorrhoid is a varicose dilation of a vein of the superior or inferior hemorrhoidal plexus, resulting from a persistent increase in venous pressure. External hemorrhoids are below the pectinate line (inferior plexus). Internal hemorrhoids are above the pectinate line (superior plexus) (Fig. 1-21).

ETIOLOGY • Low-fiber, high-fat diet • Chronic constipation and straining with defecation • High resting anal sphincter pressures • Obesity • Rectal surgery (i.e., episiotomy) • Prolonged sitting • Anal intercourse

DIAGNOSIS

SYNONYMS

DIFFERENTIAL DIAGNOSIS

Piles

• • • • • • • •

ICD-9CM CODES 455.6 Hemorrhoids

EPIDEMIOLOGY & DEMOGRAPHICS Potential for development of symptomatic hemorrhoids in all adults PREVALENCE: Estimated 50% of the adult population in the U.S. PREDOMINANT SEX: Males  females

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Painless bleeding with defecation; bleeding is bright red and staining on toilet paper • Perianal irritation • Mucofecal staining of underclothes • Acute external hemorrhoids: painful, swollen, and often thrombosed • Pain on sitting, standing, or defecating (thrombosed hemorrhoid) • Prolapse • Constipation

Fissure Abscess Anal fistula Condylomata acuminata Hypertrophied anal papillae Rectal prolapse Rectal polyp Neoplasm

WORKUP • • • •

Inspection Digital rectal examination Anoscopy Sigmoidoscopy

TREATMENT NONPHARMACOLOGIC THERAPY • Avoidance of constipation and straining with defecation • Avoidance of prolonged sitting on toilet • High-fiber diet (20 to 30 g/day) • Increased fluid intake (six to eight glasses of water per day)

• Cleaning with mild soap and water after defecation • Warm soaks or ice to soothe • Sitz baths

ACUTE GENERAL Rx • Fiber supplements to provide bulk (psyllium extracts or mucilloids) • Medicated compresses with witch hazel • Topical hydrocortisone (1% to 3% cream or ointment) • Topical anesthetic spray • Glycerin suppositories • Stool softeners • Surgically remove during first 72 hr after onset

CHRONIC Rx • • • • • • • •

Rubber-band ligation Injection sclerotherapy Photocoagulation Cryodestruction Hemorrhoidectomy Anal dilation Laser or cautery hemorrhoidectomy Observance for complications: thrombosis, bleeding, infection, anal stenosis or weakness

DISPOSITION Should resolve, but there is a high rate of recurrence

REFERRAL To colorectal or general surgeon for any hemorrhoid that does not respond to conservative therapy

PEARLS & CONSIDERATIONS COMMENTS • Patients need to understand the importance of a healthy diet, regular exercise, and rectal hygiene. • Stress the importance of avoiding prolonged sitting and straining on the toilet. • Stress the need not to defer the urge to defecate.

SUGGESTED READING Zuber TJ: Hemorrhoidectomy for thrombosed external hemorrhoids, Am Fam Physician 65:1629, 2002. AUTHOR: MARIA A. CORIGLIANO, M.D. FIGURE 1-21 Anatomy of internal and external hemorrhoids. (From Noble J [ed]: Textbook of primary care medicine, ed 2, St Louis, 1996, Mosby.)

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Herpes Zoster ETIOLOGY

DEFINITION

Reactivation of varicella virus (human herpesvirus III)

SYNONYMS Shingles ICD-9CM CODES 053.9 Herpes zoster

EPIDEMIOLOGY & DEMOGRAPHICS • Herpes zoster occurs during lifetime in 10% to 20% of the population. • There is an increased incidence in immunocompromised patients (AIDS, malignancy) and the elderly.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Pain generally precedes skin manifestation by 3 to 5 days and is generally localized to the dermatome that will be affected by the skin lesions. • Constitutional symptoms are often present (malaise, fever, headache). • The initial rash consists of erythematous maculopapules generally affecting one dermatome (thoracic region in majority of cases); some patients (50%) may have scattered vesicles outside of the affected dermatome. • The initial maculopapules evolve into vesicles and pustules by the third or the fourth day. • The vesicles have an erythematous base, are cloudy, and have various sizes (a distinguishing characteristic from herpes simplex in which the vesicles are of uniform size). • The vesicles subsequently become umbilicated and then form crusts that generally fall off within 3 wk; scarring may occur. • Pain during and after the rash is generally significant. • Secondary bacterial infection with Staphylococcus aureus or Streptococcus pyogenes may occur. • Regional lymphadenopathy may occur. • Herpes zoster may involve the trigeminal nerve (most frequent cranial nerve involved); involvement of the geniculate ganglion can cause facial palsy and a painful ear, with the presence of vesicles on the pinna and external auditory canal (Ramsay Hunt syndrome).

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Rash: herpes simplex and other viral infections • Pain from herpes zoster: may be confused with acute myocardial infarction, pulmonary embolism, pleuritis, pericarditis, renal colic, etc.

LABORATORY TESTS Laboratory tests are generally not necessary (viral cultures and Tzanck smear will confirm diagnosis in patients with atypical presentation).

TREATMENT NONPHARMACOLOGIC THERAPY • Wet compresses (using Burow’s solution or cool tap water) applied for 15 to 30 min 5 to 10 times a day are useful to break vesicles and remove serum and crust. • Care must be taken to prevent any secondary bacterial infection.

ACUTE GENERAL Rx • Gabapentin 300 to 1800 mg qd is effective in the treatment of pain and sleep interference associated with postherpetic neuralgia. • Lidocaine patch 5% (Lidoderm) is also effective in relieving postherpetic neuralgia. Patches are applied to intact skin to cover the most painful area for up to 12 hr within a 24-hr period. • Oral antiviral agents can decrease acute pain, inflammation, and vesicle formation when treatment is begun within 48 hr of onset of rash. Treatment options are: 1. Acyclovir (Zovirax) 800 mg 5 times daily for 7 to 10 days 2. Valacyclovir (Valtrex) 1000 mg tid for 7 days 3. Famciclovir (Famvir) 500 mg tid for 7 days • Immunocompromised patients should be treated with IV acyclovir 500 mg/m2 or 10 mg/kg q8h in 1-hr infusions for 7 days, with close monitoring of renal function and adequate hydration; vidarabine (continuous 12-hr infusion of 10 mg/kg/day for 7 days) is also effective for treatment of disseminated herpes zoster in immunocompromised hosts.

DISPOSITION • The incidence of postherpetic neuralgia (defined as pain that persists more than 30 days after onset of rash) increases with age (70% by age 70 yr); antivirals reduce the risk of postherpetic neuralgia. • Incidence of disseminated herpes zoster is increased in immunocompromised hosts (e.g., 15% to 50% of patients with active Hodgkin’s disease). • Immunocompromised hosts are also more prone to neurologic complications (encephalitis, myelitis, cranial and peripheral nerve palsies, acute retinal necrosis). The mortality rate is 10% to 20% in immunocompromised hosts with disseminated zoster. • Motor neuropathies occur in 5% of all cases of zoster; complete recovery occurs in 70% of patients.

REFERRAL • Hospitalization for IV acyclovir in patients with disseminated herpes zoster • Patients with herpes zoster ophthalmicus should be referred to an ophthalmologist • Surgical referral for rhizotomy in patients with severe pain unresponsive to conventional treatment • Sympathetic blocks in selected patients

SUGGESTED READING Gnann JW, Whitley RJ: Herpes zoster, N Engl J Med 347:340, 2002. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Herpes zoster is a disease caused by reactivation of the varicella-zoster virus. Following the primary infection (chickenpox) the virus becomes latent in the dorsal root ganglia and reemerges when there is a weakening of the immune system (secondary to disease or advanced age).

• Patients with AIDS and transplant patients may develop acyclovir-resistant varicella-zoster; these patients can be treated with foscarnet (40 mg/kg IV q8h) continued for at least 10 days or until lesions are completely healed. • Capsaicin cream (Zostrix) can be useful for treatment of postherpetic neuralgia. It is generally applied three times daily for several weeks after the crusts have fallen off. • Sympathetic blocks (stellate ganglion or epidural) with 0.25% bupivacaine and rhizotomy are reserved for severe cases unresponsive to conservative treatment. • Corticosteroids should be considered in older patients if there are no contraindications. Initial dose is prednisone 60 mg/day tapered over a period of 21 days. When used there is a decrease in the use of analgesics and time to resumption of usual activities, but there is no effect on the incidence and duration of postherpetic neuralgia.

Section I

BASIC INFORMATION

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Hypercholesterolemia BASIC INFORMATION DEFINITION

8. Extrahepatic biliary obstruction 9. Primary biliary cirrhosis •

Hypercholesterolemia refers to a blood cholesterol measurement 200 mg/dl. A cholesterol level of 200 to 239 mg/dl is considered borderline high, and a level of 240 mg/dl is considered to be a high cholesterol measurement.

DIAGNOSIS

SYNONYMS

LABORATORY TESTS

Hypercholesteremia

PRIMARY PREVENTION WITHOUT ATHEROSCLEROSIS OR DIABETES MELLITUS: 1. Total cholesterol 200 mg/dl, and the HDL 40: repeat in 5 yr. 2. Cholesterol 200 to 239 mg/dl and the HDL 40: discuss dietary modification, repeat in 1 to 2 yr. 3. Total cholesterol 240 mg/dl or the HDL 40 mg/dl: need a fasting lipid profile (cholesterol, HDL, triglycerides, from which an LDL can be calculated). 4. Fasting lipid profile with LDL 130 mg/dl and 1 or no risk factors: dietary guidance and repeat in 5 yr. 5. Fasting lipid profile with LDL 130 to 159 mg/dl (borderline high risk), and less than two risk factors for CAD: diet and exercise modification, with repeat profile in 12 wk. 6. Fasting lipid profile with LDL 160 mg/dl or borderline LDL and two or more risk factors for CAD: need drug therapy. SECONDARY PREVENTION WITH ATHEROSCLEROSIS OR DIABETES MELLITUS: 1. All patients: fasting lipid profile 2. If LDL 100 mg/dl: instruction on diet and exercise, and repeat after 3 to 4 months 3. Now classified as very high risk 4. If LDL 70, drug therapy advised

ICD-9CM CODES 272.0 Hypercholesterolemia

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE/PREVALENCE: • There are well over 100 million Americans with a total serum cholesterol 200 mg/dl. • Elevated cholesterol requires drug therapy in about 60 million Americans. • Incidence of heterozygous familial hypercholesterolemia: about 1:500. • Incidence of homozygous familial hypercholesterolemia: about 1:1 million. • Prevalence of hypercholesterolemia increases with increasing age. GENETICS: • Familial hypercholesterolemia: autosomal dominant disorder • Familial combined hyperlipidemia: possibly an autosomal dominant disorder • Multifactorial predilection: apparent in majority of affected individuals RISK FACTORS: • Dietary intake • Genetic predisposition • Sedentary lifestyle • Associated secondary causes • Obesity

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Most patients: no physical findings • Possible findings particularly in the familial forms 1. Tendon xanthomas 2. Xanthelasma 3. Arcus corneae 4. Coronary artery disease 5. Cerebrovascular disease 6. Peripheral arterial disease

ETIOLOGY Primary 1. Genetics 2. Obesity 3. Dietary intake Secondary 1. Diabetes mellitus 2. Alcohol 3. Hypothyroidism 4. Glucocorticoid use 5. Most diuretics 6. Nephrotic syndrome 7. Hepatoma

DIFFERENTIAL DIAGNOSIS No real differential diagnosis; however, consider underlying secondary causes/ etiologies for the elevated cholesterol.

TREATMENT NONPHARMACOLOGIC THERAPY • Dietary modifications 1. Low-cholesterol, low-fat diet (fat intake to 30% or less of the total caloric intake) 2. Saturated fats 7% of total calories 3. No more than 200 mg/day of cholesterol • Increased activity with aerobic exercise: encourage 20 to 30 min of aerobic exercise three to four times a week • Smoking cessation encouraged • Counseling on CAD risk factors

ACUTE GENERAL Rx No acute treatment needed

CHRONIC Rx • In secondary prevention: needed for patients with known CAD, vascular

•

• • • • •

disease or diabetes mellitus, and LDL 70 mg/dl In primary prevention: considered in patients on dietary therapy with LDL 190 mg/dl with no risk factors, LDL 160 mg/dl with two or more risk factors, HDL 30 mg/dl, or LDL 130 to 159 mg/dl with two or more risk factors for CAD Medications that can be used (see Table 1-7): 1. Bile acid sequestrants (poorly tolerated) 2. Niacin (poorly tolerated) 3. HMG-CoA reductase inhibitors (“statins”) 4. Fibric acids 5. Medication tailored to the patient’s lipid profile, lifestyle, and the medication’s side-effect profile Cholesterol absorption inhibitors (ezetimibe) Bile acid sequestrants to lower LDL Niacin to lower LDL and triglycerides and raise HDL HMG-CoA reductase inhibitors to lower LDL Fibric acids work to lower triglycerides more than LDL

DISPOSITION • After initiation of therapy, repeat laboratory tests in 4 to 6 wk, with modifications as necessary. • Once goal is achieved, lifelong medication and monitoring are needed at least three to four times a year. • Dietary modification is needed to continue with drug therapy. • Repeat review for additional CAD risk factors.

PEARLS & CONSIDERATIONS COMMENTS Because the rate of endpoints (coronary and cardiovascular) is higher in older patients, the absolute risk reduction is greater and the number needed to treat is lower in the geriatric patient compared with the middle-aged adult.

SUGGESTED READINGS Cleeman JI: Detection and evaluation of dyslipoproteinemia, Endocrinol Metab Clin North Am 27(3):597, 1998. Illingworth DR: Management of hypercholesterolemia, Med Clin North Am 84(1):23, 2000. National Cholesterol Education Program: Second Report on the Expert Panel on Detection, Evaluation, and Treatment of High Cholesterol in Adults (adult treatment panel IV), JAMA 285:2486, 2001. Safeer R, Ugalat P: Cholesterol treatment guidelines update, Am Fam Physician 65:871, 2002. AUTHORS: BETH J. WUTZ, M.D., with revisions by TOM J. WACHTEL, M.D.

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Hypercholesterolemia TABLE 1-7

99

Drugs Affecting Lipoprotein Metabolism

HMG-CoA reductase inhibitors (statins)

Nicotinic acid

Fibric acids

Cholesterol absorption inhibitors

Lipid/lipoprotein effects

Lovastatin (20-80 mg) Pravastatin (20-80 mg) Simvastatin (20-80 mg) Fluvastatin (20-80 mg) Atorvastatin (10-80 mg) Rosuvastain (5-40 mg) Cholestyramine (4-16 g) Colestipol (5-20 g) Colesevelam (2.6-3.8 g)

LDL HDL TG

Immediate-release (crystalline) nicotinic acid (1.5-3 g), extended-release nicotinic acid (Niaspan) 1-2 g, sustained-release nicotinic acid (1-2 g) Gemfibrozil (600 mg bid) Fenofibrate (160 mg qd) Clofibrate (1000 mg bid)

Ezetimibe (10 mg QD)

Side effects

Contraindications

↓18%-55% ↑5%-15% ↓7%-30%

Myopathy Increased liver enzymes

LDL HDL TG

↓1.5%-30% ↑3%-5% No change or increase

Gastrointestinal distress Constipation Decreased absorption of other drugs

LDL HDL TG

↓5%-25% ↑15%-35% ↓20%-50%

Flushing Hyperglycemia Hyperuricemia (or gout) Upper GI distress Hepatotoxicity

Dyspepsia Gallstones Myopathy

Absolute: • Active or chronic liver disease Relative: • Concomitant use of certain drugs* Absolute: • Dysbetalipoproteinemia • TG 400 mg/dl Relative: • TG 200 mg/dl Absolute: • Chronic liver disease • Severe gout Relative: • Diabetes • Hyperuricemia • Peptic ulcer disease Absolute: • Severe renal disease • Severe hepatic disease

Abdominal pain myalgias

• Severe renal disease • Severe hepatic disease

LDL ↓5%-20% (may be increased in patients with high TG) HDL ↑10%-20% TG ↓20%-50% LDL ↓18% HDL ↑1% TG ↓7%-8%

Modified from The National Cholesterol Education Program, JAMA 285:2486, 2001. CoA, Coenzyme A; GI, gastrointestinal; HDL, high-density lipoprotein; HMG, 3-hydroxy-3 methylglutaryl; LDL, low-density lipoprotein; TG, triglyceride. *Cyclosporine, macrolide antibiotics, various antifungal agents, and cytochrome P-450 inhibitors (fibrates and niacin should be used with appropriate caution).

DISEASES AND DISORDERS

Bile acid sequestrants

Agents and daily doses

Section I

Drug class

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Hyperosmolar Coma

DIFFERENTIAL DIAGNOSIS

• Monitor mental status, vital signs, urine output qh until improved, then monitor q2-4h. • Monitor electrolytes, renal function, and glucose level (see Acute General Rx).

12 hr). Serum KCl replacement in patients with normal renal function and adequate urinary output is started when the serum potassium level is 5.2 mEq/L (e.g., 10 mEq KCl/hr if potassium level is 4 to 5.2 mEq/L). Continuous ECG monitoring and hourly measurement of urinary output are recommended. • Correct hyperglycemia. The goal is for plasma glucose to decline by at least 75 to 100 mg/dl/hr. 1. Vigorous IV hydration will decrease the serum glucose level in most patients by 80 mg/dl/hr; a regular insulin IV bolus (10 U) is often not necessary. 2. Low-dose insulin infusion at 1 to 2 U/hr (e.g., 25 U of regular insulin in 250 ml of 0.9% saline solution at 20 ml/hr) until the serum glucose level approaches 300 mg/dl; then the patient is started on regular SC insulin with sliding scale coverage. If the plasma glucose does not decrease over 2 to 4 hr despite adequate fluid administration and urine output, consider doubling the hourly insulin dose. 3. Glucose should be monitored q12h in the initial 12 hr. • In the absence of renal failure, phosphate can be administered at a rate of 0.1 mmol/kg/hr (5 to 10 mmol/hr) to a maximum of 80 to 120 mmol in 24 hr. Magnesium replacement, in absence of renal failure, can be administered IM (0.05 to 0.10 ml/kg of 20% magnesium sulfate) or as IV infusion (4 to 8 ml of 20% magnesium sulfate [0.08 to 0.16 mEq/kg]). Repeat magnesium, phosphate, and calcium levels should be obtained after 12 to 24 hr.

• Diabetic ketoacidosis

ACUTE GENERAL Rx

DISPOSITION

LABORATORY TESTS

• Vigorous fluid replacement: the volume and rate of fluid replacement are determined by renal and cardiac function. Typically, infuse 1000 to 1500 ml/hr for the initial 1 to 2 L; then decrease the rate of infusion to 500 ml/hr and monitor urinary output, blood chemistries, and blood pressure; use 0.9% NS (isotonic solution) if the patient is hypotensive; otherwise use 0.45% NS solution. Slower infusion rate may be used initially in patients with compromised cardiovascular or renal status. • Replace electrolytes and monitor serum levels frequently (e.g., serum sodium and potassium q2h for the first

BASIC INFORMATION DEFINITION Hyperosmolar coma (nonketotic hyperosmolar syndrome) is a state of extreme hyperglycemia, marked dehydration, serum hyperosmolarity, altered mental status, and absence of ketoacidosis.

SYNONYMS Nonketotic hyperosmolar syndrome Hyperosmolar nonketotic state ICD-9CM CODES 250.2 Hyperosmolar coma

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Evidence of severe dehydration (poor skin turgor, sunken eyeballs, dry mucous membranes) • Neurologic defects (reversible hemiplegia, focal seizures) • Orthostatic hypotension, tachycardia • Evidence of precipitating factors (pneumonia, infected skin ulcer) • Coma (25% of patients), delirium

ETIOLOGY • Infections, 20% to 25% (e.g., pneumonia, UTI, sepsis) • New or previously unrecognized diabetes (30% to 50%) • Reduction or omission of diabetic medication • Stress (MI, CVA) • Drugs: diuretics (dehydration), phenytoin, diazoxide (impaired insulin secretion)

DIAGNOSIS

• Hyperglycemia: serum glucose usually 600 mg/dl. • Hyperosmolarity: serum osmolarity usually 340 mOsm/L. • Serum sodium: may be low, normal, or high; if normal or high, the patient is severely dehydrated, because an elevated glucose draws fluid from intracellular space decreasing the serum sodium; the corrected sodium can be obtained by increasing the serum sodium concentration by 1.6 mEq/dl for every 100 mg/dl increase in the serum glucose level over normal.

• Serum potassium: may be low, normal, or high; regardless of the initial serum level, the total body deficit is approximately 5 to 15 mEq/kg. • Serum bicarbonate: usually 12 mEq/L (average is 17 mEq/L). • Arterial pH: usually 7.2 (average is 7.26); both serum bicarbonate and arterial pH may be lower if lactic acidosis is present. • BUN: azotemia (prerenal) is usually present (BUN generally ranges from 60 to 90 mg/dl). • Phosphorus: hypophosphatemia (average deficit is 70 to 140 mEq). • Calcium: hypocalcemia (average deficit is 50 to 100 mEq). • Magnesium: hypomagnesemia (average deficit is 50 to 100 mEq). • CBC with differential, urinalysis, blood and urine cultures should be performed to rule out infectious etiology.

IMAGING STUDIES • Chest x-ray examination is useful to rule out infectious process. The initial chest x-ray may be negative if the patient has significant dehydration. Repeat chest x-ray examination after 24 hr of hydration if pulmonary infection is suspected. • CT scan of head should be performed in patients with suspected CVA.

TREATMENT NONPHARMACOLOGIC THERAPY

Mortality in nonketotic hyperosmolar coma ranges from 20% to 50%.

PEARLS & CONSIDERATIONS COMMENTS The typical patient presenting with hyperosmolar coma is an elderly or bedconfined diabetic with impaired ability to communicate thirst who is evaluated after an interval of 1 to 2 wk of prolonged osmotic diuresis. AUTHOR: FRED F. FERRI, M.D.

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Hyperparathyroidism

DEFINITION

DIFFERENTIAL DIAGNOSIS

Primary hyperparathyroidism is an endocrine disorder caused by the excessive secretion of parathyroid hormone (PTH) from the parathyroid glands.

Other causes of hypercalcemia: • Malignancy: neoplasms of breast, lung, kidney, ovary, pancreas; myeloma, lymphoma • Granulomatous disorders (e.g., sarcoidosis) • Paget’s disease • Vitamin D intoxication, milk-alkali syndrome • Thiazide diuretics • Other: familial hypocalciuric hypercalcemia, thyrotoxicosis, adrenal insufficiency, prolonged immobilization, vitamin A intoxication, recovery from acute renal failure, lithium administration, pheochromocytoma, SLE

ICD-9CM CODES 252.0 Primary hyperparathyroidism 588.8 Secondary hyperparathyroidism in chronic renal disease

EPIDEMIOLOGY & DEMOGRAPHICS PREDOMINANT AGE AND SEX: • Primary hyperparathyroidism occurs most frequently in postmenopausal women; prevalence in this group may be as high as 3%. The condition is asymptomatic in 50% of patients • Prevalence: 1 case/1000 men and 2 to 3 cases/1000 women • Primary hyperparathyroidism is the most frequent cause of hypercalcemia in ambulatory patients whereas malignancy is the most frequent cause of hypercalcemia in hospitalized patients. GENETICS: Hyperparathyroidism can occur in conjunction with MEN I or II.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Primary hyperparathyroidism can be classified as asymptomatic (75% to 80%) and symptomatic. Physical examination may be entirely normal. The presence of signs and symptoms varies with the rapidity of development and degree of hypercalcemia. The following abnormalities may be present: • GI: constipation, anorexia, nausea, vomiting, pancreatitis, ulcers • CNS: confusion, obtundation, psychosis, lassitude, depression, coma • GU: nephrolithiasis, renal insufficiency, polyuria, decreased urine-concentrating ability, nocturia, nephrocalcinosis • Musculoskeletal: myopathy, weakness, osteoporosis, pseudogout, bone pain • Other: hypertension, metastatic calcifications, band keratopathy (found in medial and lateral margin of the cornea), pruritus

ETIOLOGY • A single adenoma is found in 80% of patients; 90% of the adenomas are found within one of the parathyroid glands, the other 10% are in ectopic sites (lateral neck, thyroid, mediastinum, retroesophagus). • Parathyroid gland hyperplasia occurs in 20% of patients. • Primary hyperthyroidism is associated with multiple endocrine neoplasia (MEN) I and II.

WORKUP • Persistent hypercalcemia, hypophosphatemia, and an elevated serum PTH confirm the diagnosis of primary hyperparathyroidism. Repeated measurements of serum calcium may be necessary because patients may not have persistently elevated serum calcium level. In malnourished patients, the serum calcium level needs to be corrected for low albumin levels by adding 0.8 mg/dl to the total serum calcium level for every 1.0 g/dl by which the serum albumin concentration is lower than 4 g/dl. • The serum PTH level is the single best test for initial evaluation of confirmed hypercalcemia. The “intact” PTH (iPTH) is the best assay. The iPTH distinguishes primary hyperparathyroidism from hypercalcemia caused by malignancy when the serum calcium level is 12 mg/dl. • A high level of urinary cyclic AMP is also suggestive of primary hyperparathyroidism. • Parathyroid hormone–like protein (PLP) is increased in hypercalcemia associated with solid malignancies. • ECG may reveal shortening of the QT interval secondary to hypercalcemia.

LABORATORY TESTS • Elevated serum ionized calcium level, low serum phosphorus, and normal or elevated alkaline phosphatase • Elevated urine calcium level (in contrast with very low urinary calcium levels seen in patients with familial hypocalciuric hypercalcemia) • Possibly elevated serum chloride levels, decreased serum CO2, hyperchloremic metabolic acidosis • A serum albumin level should be obtained when measuring serum calcium and the calcium level should be adjusted (see above) in hypoalbuminemic patients

• The differential diagnosis of hypercalcemia is described in Section II

IMAGING STUDIES • A bone survey may show evidence of subperiosteal bone resorption (suggesting PTH excess). The classic bone disease of primary hyperparathyroidism is osteitis fibrosa cystica. • Parathyroid localization with technetium-99m sestamibi has been shown to have a high sensitivity and specificity for single adenomas. • Screen for osteopenia with measurement of bone mineral density in all postmenopausal women.

TREATMENT NONPHARMACOLOGIC THERAPY • Unless contraindicated, patients should maintain a high intake of fluids (3 to 5 L/day) and sodium chloride (400 mEq/day) to increase renal calcium excretion. Calcium intake should be 1000 mg/day. • Potential hypercalcemic agents (e.g., thiazide diuretics) should be discontinued. • Surgery is the only effective treatment for primary hyperparathyroidism. It is generally indicated in all female patients and male patients with complications from hyperthyroidism, such as nephrolithiasis and osteopenia. The conventional surgical approach is bilateral neck exploration under general anesthesia. Minimally invasive adenomectomy guided by preoperative technetium-99-m sestamibi scanning or ultrasound plus spiral CT is an alternative to conventional neck exploration. With the minimally invasive approach, the solitary adenoma is excised through a small unilateral incision with the patient under local cervical block anesthesia. • Percutaneous ethanol injection into the parathyroid gland should be considered in selected patients who have undergone a subtotal parathyroidectomy for multigland disease and have recurrent hyperparathyroidism as a result of remnant gland. • Asymptomatic elderly male patients can be followed conservatively with periodic monitoring of serum calcium level and review of symptoms. Serum creatinine and PTH levels should also be obtained at 6- to 12-mo intervals, bone density (cortical and trabecular) yearly. Criteria for medical monitoring of patients with asymptomatic primary hyperparathyroidism are as follows: 1. Serum calcium level only mildly elevated 2. Asymptomatic patient

DISEASES AND DISORDERS

DIAGNOSIS

Section I

BASIC INFORMATION

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Hyperparathyroidism 3. Normal bone status (no osteoporosis) 4. Normal kidney function and no urolithiasis or nephrocalcinosis 5. No previous episode of life-threatening hypercalcemia • Nearly 25% of asymptomatic patients develop indications for surgery during observation.

ACUTE GENERAL Rx Acute severe hypercalcemia (serum calcium 13 mg/dl) or symptomatic patients can be treated with the following: • Vigorous IV hydration with NS followed by IV furosemide. Use NS with caution in patients with cardiac or renal insufficiency to avoid fluid overload. • Calcitonin 4 IU/kg q12h is indicated when saline hydration and furosemide are ineffective or contraindicated. • Bisphosphonates (pamidronate, etidronate), mithramycin, and gallium

nitrate are also effective for severe hypercalcemia. • Cinacalcet (Sensipar) is an oral calcimimetic agent that directly lowers PTH levels by increasing the calciumsensing receptor to extracellular calcium. The reduction in PTH is associated with a concomitant decrease in serum calcium levels. It is indicated in treatment of secondary hyperparathyroidism in patients with chronic kidney disease on dialysis and hypercalcemia in parathyroid carcinoma. Initial dose is 30 mg po qd.

PEARLS & CONSIDERATIONS COMMENTS • Patients with hyperparathyroidism should undergo further evaluation for the presence of MEN I or II.

• Decreased bone mineral density and nephrolithiasis are the major sequelae of untreated hyperparathyroidism. • An experienced endocrine surgeon cures more than 95% of patients undergoing bilateral neck exploration and incurs 1% perioperative mortality.

SUGGESTED READINGS Monchik JM et al: Minimally invasive parathyroid surgery in 103 patients with local/regional anesthesia, without exclusion criteria, Surgery 131:502, 2002. Taniegra ED: Hyperparathyroidism, Am Fam Physician 69:333, 2004. Udelsman R: Six hundred fifty-six consecutive explorations for primary hyperparathyroidism, Ann Surg 235:665, 2002. AUTHOR: FRED F. FERRI, M.D.

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Hypertension BASIC INFORMATION

SYNONYMS Essential hypertension Idiopathic hypertension High blood pressure ICD-9CM CODES 401.1 Essential hypertension 401.0 Malignant hypertension caused by renal artery stenosis 405.01 Malignant hypertension secondary to renal artery stenosis 437.2 Hypertensive encephalopathy

EPIDEMIOLOGY & DEMOGRAPHICS • Incidence of hypertension in adult population older than age 60: 65%. • Two thirds have isolated systolic hypertension, which is no longer considered a separate clinical entity. • 90% of persons age 55 will develop hypertension in their lifetime. • 50 million individuals in the U.S. and approximately 1 billion individuals worldwide meet the criteria for diagnosis of hypertension.

ETIOLOGY

TREATMENT

• • • • • • • •

NONPHARMACOLOGIC THERAPY

Essential (primary) hypertension (90%) Drug induced or drug related (5%) Renal parenchymal disease (3%) Renovascular hypertension (2%) Endocrine (4% to 5%) Primary aldosteronism (0.5%) Pheochromocytoma (0.2%) Cushing’s syndrome and chronic steroid therapy (0.2%) • Hyperparathyroidism or thyroid disease (0.2%) • Coarctation of the aorta (0.2%)

PHYSICAL FINDINGS & CLINICAL PRESENTATION

DIAGNOSIS

Physical examination may be entirely within normal limits except for the presence of hypertension. A proper initial physical examination on a hypertensive patient should include the following: • Measure height and weight. • Evaluate skin for the presence of striae (Cushing’s syndrome). • Perform funduscopic examination: check for papilledema, retinal exudates, hemorrhages, arterial narrowing, AV compression. • Examine the neck for carotid bruits, distended neck veins, or enlarged thyroid gland. • Perform cardiopulmonary examination: check for loud aortic component of S2, S4, ventricular lift, murmurs, arrhythmias.

WORKUP Pertinent history: • Age of onset of hypertension, previous antihypertensive therapy • Family history of hypertension, stroke, cardiovascular disease • Diet, salt intake, alcohol, drugs (e.g., NSAIDs, decongestants, steroids) • Occupation, lifestyle, socioeconomic status, psychologic factors • Other cardiovascular risk factors: hyperlipidemia, obesity, diabetes mellitus, carbohydrate intolerance • Symptoms of secondary hypertension: 1. Headache, palpitations, excessive perspiration (possible pheochromocytoma) 2. Weakness, polyuria (consider hyperaldosteronism) 3. Claudication of lower extremities (seen with coarctation of aorta)

• Urinalysis: for evidence of renal disease. • BUN, creatinine: to rule out renal disease. High-serum creatinine is a predictor of cardiovascular risk in essential hypertension. • Serum electrolyte levels: low potassium is suggestive of primary aldosteronism, diuretic use, or Cushing’s syndrome. • Fasting serum glucose to screen for Cushing’s syndrome. • Screening for coexisting diseases that may adversely affect prognosis: 1. Serum lipid panel, uric acid, calcium 2. If pheochromocytoma is suspected: 24-hr urine for VMA and metanephrines

IMAGING STUDIES • ECG: check for presence of left ventricular hypertrophy (LVH) with strain pattern. • Echocardiogram in selected cases. • MRA of the renal arteries: in suspected renovascular hypertension (renal artery stenosis).

Lifestyle modifications: • Lose weight if overweight. • Limit alcohol intake to 1 oz of ethanol per day in men or 0.5 oz in women. • Exercise (aerobic) regularly (30 min/day, most days). • Reduce sodium intake. • Maintain adequate dietary potassium (3500 mg/day) intake. • Stop smoking and reduce dietary saturated fat and cholesterol intake for overall cardiovascular health. • Consume diet rich in fruits and vegetables.

ACUTE GENERAL Rx According to the Seventh Report of the Joint National Committee on Detection, Evaluation, and Treatment of High Blood Pressure: • Antihypertensive drug therapy should be initiated in patients with stage 1 hypertension. Diuretics or -blockers are preferred for initial therapy because a reduction in morbidity and mortality has been demonstrated and because of their lower cost. • ACE inhibitors, ARBs, calcium channel blockers, and --blockers are also effective. • Two-drug combination is necessary for most patients with stage 2 hypertension. Fixed dose combinations may improve compliance and cut cost.

DISEASES AND DISORDERS

The Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC 7) classifies normal blood pressure in adults as 120 mm Hg systolic and 80 mm Hg diastolic. Prehypertension is defined as systolic pressure 120 to 139 mm Hg or diastolic pressure 80 to 89 mm Hg. Stage 1 hypertension is systolic BP 140 to 159 mm Hg or diastolic BP 90 to 99 mm Hg. Stage 2 hypertension is systolic BP 160 mm Hg or diastolic BP 100 mm Hg.

LABORATORY TESTS Section I

DEFINITION

• Check abdomen for masses (pheochromocytoma, polycystic kidneys), presence of bruits over the renal artery (renal artery stenosis), dilation of the aorta. • Obtain two or more BP measurements separated by 2 min with the patient either supine or seated and after standing for at least 2 min (the latter to check for orthostatic hypotension). Measure BP in both upper extremities (if values are discrepant, use the higher value). Blood should be measured at least twice (on separate visits) in the sitting position before making a diagnosis of hypertension. • Examine arterial pulses (dilated or absent femoral pulses and BP greater in upper extremities than lower extremities suggest aortic coarctation). • Note the presence of truncal obesity (Cushing’s syndrome) and pedal edema (CHF, nephrosis). • Perform neurologic assessment. • The clinical evaluation should help determine if the patient has primary or secondary (possibly reversible) hypertension, if there is target organ disease present, and if there are cardiovascular risk factors in addition to hypertension.

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Hypertension • The major advantages and limitations of each class of drugs are described as follows: 1. Diuretics a. Advantages: inexpensive, once per day dosing. Useful in edema states, CHF, chronic renal disease (decreased incidence of hip fractures in patients taking thiazide diuretics; opposite effect for loop diuretics). b. Disadvantages: significant adverse metabolic effects, increased risk of cardiac arrhythmias, sexual dysfunction, possible adverse effects on lipids and glucose levels. Avoid in patients with urinary incontinence. Thiazides not effective when creatinine clearance is less than 20 ml/min. (Note: low creatinine clearance can coexist with normal creatinine levels in patients over the age of 85 [see Section IV].) 2. -blockers a. Advantages: ideal in hypertensive patients with ischemic heart disease or post-MI. Favored in hyperkinetic, younger patients (resting tachycardia, wide pulse pressure, hyperdynamic heart) and stable (Class II-III) CHF patients. b. Disadvantages: adverse effect on quality of life (increased incidence of fatigue, depression, impotence, bronchospasm, hypoglycemia, peripheral vascular disease, adverse effects on lipids, masking of signs and symptoms of hypoglycemia in diabetics). 3. Calcium channel blockers a. Advantages: helpful in hypertensive patients with ischemic heart disease. Generally favorable effect on quality of life; can be used in patients with bronchospastic disorders, renal disease, peripheral avascular disease, metabolic disorders, and salt sensitivity. Nondihydropyridine calcium channel blockers (verapamil, diltiazem) are useful in reducing proteinuria. b. Disadvantages: diltiazem and verapamil should be avoided in patients with CHF because of their negative chronotropic and inotropic effects; pedal edema may occur with nifedipine and amlodipine; constipation can be a problem in patients receiving verapamil.

4. ACE inhibitors a. Advantages: well tolerated, favorable impact on quality of life; useful in hypertension complicated by CHF; helpful in prevention of diabetic renal disease; effective in decreasing LVH. b. Disadvantages: cough is a frequent side effect (5% to 20% of patients); hyperkalemia may occur in patients with diabetes or renal insufficiency; hypotension may occur in volumedepleted patients. 5. Angiotensin II receptor blockers (ARB) a. Advantages: well tolerated, favorable impact on quality of life; useful in patients unable to tolerate ACE inhibitors because of persistent cough and in CHF and diabetic patients; single daily dose. b. Disadvantages: cost; hypotension may occur in volume-depleted patients. 6. -1 adrenergic blockers a. Advantages: no adverse effect on blood lipids or insulin sensitivity; helpful in BPH. May be less effective than other agents. b. Disadvantages: postural hypotension; syncope can be avoided by giving an initial low dose at bedtime. 7. Other agents (e.g., hydralazine, reserpine, guanfacine, methyldopa, minoxidil, clonidine) are infrequently used. 8. Start with a low dose and titrate upward slowly, monitoring for orthostatic hypotension. Consider titrating down if BP is consistently below 130/80. TREATMENT OF RENOVASCULAR HYPERTENSION (RVH): Therapeutic approach varies with the cause of the RVH. 1. Medical therapy is advisable in elderly patients with atheromatous renal vascular hypertension; useful agents are: a. -blockers: very effective in patients with elevated plasma renin. b. ACE inhibitors: very effective; however, should be avoided in patients with bilateral renal artery stenosis or in patients with solitary kidney and renal stenosis. c. Diuretics: often used in combination with ACE inhibitors. 2. Surgical revascularization is generally reserved for atheromatous RVH in patients responding poorly to medical therapy (uncontrolled hypertension, deteriorating renal function).

MALIGNANT HYPERTENSION, HYPERTENSIVE EMERGENCIES, AND HYPERTENSIVE URGENCIES • Definitions: 1. Malignant hypertension is a potentially life-threatening situation. a. The rate of BP rise is a critical factor. b. The clinical manifestations are grade IV hypertensive retinopathy (exudates, hemorrhages, and papilledema), cardiovascular or renal compromise, and encephalopathy. c. It requires rapid BP reduction (not necessarily into normal ranges) to prevent or limit target organ disease. 2. Hypertensive emergencies are situations that require immediate (within 1 hr) lowering of BP to prevent end-organ damage. The choice of therapeutic agents in malignant hypertension varies with the cause. 1. Nitroprusside is the drug of choice in hypertensive encephalopathy, hypertension and intracranial bleeding, malignant hypertension, hypertension and heart failure, dissecting aortic aneurysm (used in combination with the propranolol); its onset of action is immediate. 2. Fenoldopam is a newer vasodilator agent useful for the short-term (up to 48 hr) management of severe hypertension when rapid but quickly reversible reduction of blood pressure is required. 3. The following are important points to remember when treating hypertensive emergencies: a. Introduce a plan for long-term therapy at the time of the initial emergency treatment. b. Agents that reduce arterial pressure can cause the kidney to retain sodium and water; therefore, the judicious administration of diuretics should accompany their use. c. The initial goal of antihypertensive therapy is not to achieve a normal BP, but rather to gradually reduce the BP; cerebral hyperperfusion may occur if the mean BP is lowered 40% in the initial 24 hr. d. Hypertensive emergencies can be treated with oral clonidine 0.1 mg q20 min (to a maximum of 0.8 mg) or intravenous medication.

PEARLS & CONSIDERATIONS COMMENTS • In patients with hypertension and chronic renal insufficiency, it is not uncommon to see a small rise in serum

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Hypertension SUGGESTED READINGS

Magill MK et al: New developments in the management of hypertension, Am Fam Physician 68:853, 2003. Malacco E et al: Treatment of isolated systolic hypertension: the SHELL study results, Blood Press 12:160-167, 2003. Oparil S et al: Pathogenesis of hypertension, Ann Intern Med 139:761, 2003. Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure, JAMA 289:2560, 2003. Staessen JA et al: Risks of untreated and treated isolated systolic hypertension in the elderly: meta-analysis of outcome trials (published erratum appears in Lancet 357:724, 2001), Lancet 355:865-872, 2000. Wing LM et al: A comparison of outcomes with angiotensin-converting-enzyme inhibitors and diuretics for hypertension in the elderly, N Engl J Med 348:583-592, 2003. Wright JT et al: Outcomes in hypertensive black and non-black patients, JAMA 293:1595-1608, 2005. AUTHORS: FRED F. FERRI, M.D., and TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

ALLHAT Officers and Coordinators for the ALLHAT Collaborative Research Group: Major outcomes in high-risk hypertensive patients randomized to angiotensin-converting enzyme inhibitor of calcium channel blocker vs diuretic: the Antihypertensive and Lipid-Lowering Treatment to Prevent Heart Attack Trial (ALLHAT) (published erratum appears in JAMA 289:178, 2003), JAMA 288:2981-2997, 2002. Aronow WS: Treatment of older persons with hypertension, Clin Geriatrics 13:12-16, 2005. Dickerson LM, Gibson MV: Management of hypertension in older persons, Am Fam Physician 71:469-476, 2005. Francos GC, Schairer HL Jr.: Hypertension. Contemporary challenges in geriatric care, Geriatrics 58:44-49, 2003. Franklin SS et al: Predominance of isolated systolic hypertension among middle-aged and elderly U.S. hypertensives: analysis based on National Health and Nutrition Examination Survey (NHANES) III, Hypertension 37:869-874, 2001. Gueyffier F et al: Antihypertensive drugs in very old people: a subgroup meta-analysis of randomized controlled trials, Lancet 353:793-796, 1999.

Section I

creatinine as the blood pressure is lowered. Most physicians will respond by decreasing the dose of the antihypertensive medication. This approach should be discouraged because it is not optimal for the long-term preservation of renal function because a small, nonprogressive increase in serum creatinine in the context of improved blood pressure control is indicative of successful reduction of the intraglomerular pressure. • The choice of antihypertensive treatment should take comorbidities into account particularly in older patients who are at risk of polypharmacy. • Home blood pressure monitoring (e.g., once weekly) with a digital measuring device is gaining in popularity. The device should be checked against the physician’s equipment from time to time. A blood pressure log should be maintained by the patient. This strategy eliminates “white coat” hypertension and provides a better sampling of BP measurements. However, no one knows what are optimal home blood pressures.

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Hyperthyroidism BASIC INFORMATION DEFINITION Hyperthyroidism is a hypermetabolic state resulting from excess thyroid hormone.

SYNONYMS Thyrotoxicosis ICD-9CM CODES 242.9 Hyperthyroidism 242.0 Hyperthyroidism with goiter 242.2 Hyperthyroidism, multinodular 242.3 Hyperthyroidism, uninodular

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE/PREVALENCE: • Hyperthyroidism affects 2% of women and 0.2% of men in their lifetime. • Toxic multinodular goiter usually occurs in women 55 yr old and is more common than Graves’ disease in the elderly.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients with hyperthyroidism generally present with the following clinical manifestations: tachycardia, tremor, hyperreflexia, anxiety, irritability, emotional lability, panic attacks, heat intolerance, sweating, increased appetite, diarrhea, weight loss; the presentation may be different in elderly patients (see third bullet).

• Patients with Graves’ disease may present with exophthalmos, lid retraction (Fig. 1-22, A), lid lag (Graves’ ophthalmopathy). The following signs and symptoms of ophthalmopathy may be present: blurring of vision, photophobia, increased lacrimation, double vision, deep orbital pressure. Clubbing of fingers associated with periosteal new bone formation in other skeletal areas (Graves’ acropachy) and pretibial myxedema (Fig. 1-22, B) may also be noted. • In the elderly the clinical signs of hyperthyroidism may be masked by manifestations of coexisting disease (e.g., new-onset atrial fibrillation, exacerbation of CHF) or may present with failure to thrive (apathetic hyperthyroidism) or dementia.

ETIOLOGY • Graves’ disease (diffuse toxic goiter) • Toxic multinodular goiter (Plummer’s disease) • Toxic adenoma • Iatrogenic and factitious • Transient hyperthyroidism (subacute thyroiditis, Hashimoto’s thyroiditis) • Rare causes: hypersecretion of TSH (e.g., pituitary neoplasms), struma ovarii, ingestion of large amount of iodine in a patient with preexisting thyroid hyperplasia or adenoma (JodBasedow phenomenon), carcinoma of thyroid, amiodarone therapy

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Anxiety disorder Pheochromocytoma Metastatic neoplasm Diabetes mellitus

WORKUP Suspected hyperthyroidism requires laboratory confirmation and identification of its etiology, because treatment varies with its cause. A detailed medical history will often provide clues to the diagnosis and etiology of the hyperthyroidism.

LABORATORY TESTS • Elevated free thyroxine (T4) • Elevated free triiodothyronine (T3): generally not necessary for diagnosis • Low TSH (unless hyperthyroidism is a result of the rare hypersecretion of TSH from a pituitary adenoma) • Thyroid autoantibodies useful in selected cases to differentiate Graves’ disease from toxic multinodular goiter (absent thyroid antibodies)

IMAGING STUDIES • 24-hr radioactive iodine uptake (RAIU) is useful to distinguish hyperthyroidism from iatrogenic thyroid hormone synthesis (thyrotoxicosis factitia) and from thyroiditis. • An overactive thyroid shows increased uptake, whereas a normal underactive

A

B

FIGURE 1-22 A, Unilateral (left) lid retraction in a patient with hyperthyroidism. B, Pretibial myxedema (arrows) in a patient with Graves’ disease. (From Noble J [ed]: Textbook of primary care medicine, ed 2, St Louis, 1996, Mosby.)

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Hyperthyroidism

NONPHARMACOLOGIC THERAPY Patient education regarding thyroid disease and discussion of the therapeutic options (medications, radioactive iodine, and thyroid surgery)

ACUTE GENERAL Rx ANTITHYROID DRUGS (THIONAMIDES): Propylthiouracil (PTU) and methimazole (Tapazole) inhibit thyroid hormone synthesis by blocking production of thyroid peroxidase (PTU and methimazole) or inhibit peripheral conversion of T4 to T3 (PTU). 1. Dosage: PTU 50 to 100 mg PO q8h; methimazole 10 to 20 mg PO q8h or 30 to 60 mg/day given as a single dose. 2. Antithyroid drugs can be used as the primary form of treatment or as adjunctive therapy before radioactive therapy or surgery or afterward if the hyperthyroidism recurs. 3. Side effects: skin rash (3% to 5% of patients), arthralgias, myalgias, granulocytopenia (0.5%). Rare side effects are aplastic anemia, hepatic necrosis from PTU, cholestatic jaundice from methimazole. 4. When antithyroid drugs are used as primary therapy, they are usually given for 6 to 24 mo; prolonged therapy may cause hypothyroidism. 5. The use of antithyroid drugs before radioactive iodine therapy is best reserved for patients in whom exacerbation of hyperthyroidism after radioactive iodine therapy is hazardous (e.g., elderly patients with coronary

CHRONIC Rx Patients undergoing treatment with antithyroid drugs should be seen every 1 to 3 mo until euthyroidism is achieved and every 3 to 4 mo while they remain on antithyroid therapy. After treatment is stopped, periodic monitoring of thyroid function tests with TSH every 3 mo for 1 yr, then every 6 mo for 1 yr, then annually is recommended.

DISPOSITION Successful treatment of hyperthyroidism requires lifelong monitoring for the onset of hypothyroidism or the recurrence of thyrotoxicosis.

REFERRAL • Endocrinology referral is recommended at the time of initial diagnosis and during treatment • Surgical referral in selected patients (see Surgical Therapy) • Hospitalization of all patients with thyroid storm

PEARLS & CONSIDERATIONS COMMENTS • Elderly hyperthyroid patients may have only subtle signs (weight loss, tachycardia, fine skin, brittle nails). This form is known as apathetic hyperthyroidism and manifests with lethargy rather than hyperkinetic activity. An enlarged thyroid gland may be absent. Coexisting medical disorders (most commonly cardiac disease) may also mask the symptoms. These patients often have unexplained CHF, worsening of angina, or new-onset atrial fibrillation resistant to treatment. • Subclinical hyperthyroidism is defined as a normal serum free thyroxine and free triiodothyronine levels with a thyroid-stimulating hormone level suppressed below the normal range and usually undetectable. These patients usually do not present with signs or symptoms of overt hyperthyroidism. Treatment options include observation or a therapeutic trial of low-dose antithyroid agents for 6 mo to attempt to induce remission.

SUGGESTED READINGS Kearns AE, Thompson GB: Medical and surgical management of hyperthyroidism, Mayo Clin Proc 77:87, 2002. Shrier DK et al: Subclinical hyperthyroidism: controversies in management, Am Fam Physician 65:431, 2002. Toft AD: Subclinical hyperthyroidism, N Engl J Med 345:512, 2001. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

TREATMENT

artery disease or significant coexisting morbidity). In these patients the antithyroid drug can be stopped 2 days before radioactive iodine therapy, resumed 2 days later, and continued for 4 to 6 wk. RADIOACTIVE IODINE (RAI; 131I): 1. RAI is the treatment of choice for patients with Graves’ disease. Radioiodine is also used in hyperthyroidism caused by toxic adenoma or toxic multinodular goiter. 2. A single dose of radioactive iodine is effective in inducing euthyroid state in nearly 80% of patients. 3. There is a high incidence of postradioactive iodine hypothyroidism (50% within first year and 2%/yr thereafter); therefore these patients should be frequently evaluated for the onset of hypothyroidism (see Chronic Rx). SURGICAL THERAPY (SUBTOTAL THYROIDECTOMY): 1. Indicated in obstructing goiters and in any patient who refuses radioactive iodine and cannot be adequately managed with antithyroid medications (e.g., patients with toxic adenoma or toxic multinodular goiter). 2. Patients should be rendered euthyroid with antithyroid drugs before surgery. 3. Complications of surgery include hypothyroidism (28% to 43% after 10 yr), hypoparathyroidism, and vocal cord paralysis (1%). 4. Hyperthyroidism recurs after surgery in 10% to 15% of patients. ADJUNCTIVE THERAPY: Propranolol alleviates the -adrenergic symptoms of hyperthyroidism; initial dose is 20 to 40 mg PO q6h; dosage is gradually increased until symptoms are controlled; major contraindications to use of propranolol are CHF and bronchospasm.

Section I

thyroid (iatrogenic thyroid ingestion, painless or subacute thyroiditis) shows normal or decreased uptake. • The RAIU results also vary with the etiology of the hyperthyroidism: Graves’ disease: increased homogeneous uptake. Multinodular goiter: increased heterogeneous uptake. Hot nodule: single focus of increased uptake. • RAIU is also generally performed before the therapeutic administration of radioactive iodine to determine the appropriate dose.

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Hypothyroidism BASIC INFORMATION DEFINITION Hypothyroidism is a disorder caused by the inadequate secretion of thyroid hormone.

SYNONYMS Myxedema ICD-9CM CODES 244 Acquired hypothyroidism 244.1 Surgical hypothyroidism 244.3 Iatrogenic hypothyroidism 244.8 Pituitary hypothyroidism 246.1 Sporadic goitrous hypothyroidism

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE/PREVALENCE: 1.5% to 2% of women and 0.2% of men PREDOMINANT AGE: Incidence of hypothyroidism increases with age; among persons older than 60 yr, 6% of women and 2.5% of men have laboratory evidence of hypothyroidism (TSH  twice normal).

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Hypothyroid patients generally present with the following signs and symptoms: fatigue, lethargy, weakness, constipation, weight gain, cold intolerance, muscle weakness, slow speech, slow cerebration with poor memory. • Skin: dry, coarse, thick, cool, sallow (yellow color caused by carotenemia); nonpitting edema in skin of eyelids and hands (myxedema) secondary to infiltration of subcutaneous tissues by a hydrophilic mucopolysaccharide substance. • Hair: brittle and coarse; loss of outer one third of eyebrows. • Facies: dulled expression, thickened tongue, thick slow-moving lips. • Thyroid gland: may or may not be palpable (depending on the cause of the hypothyroidism). • Heart sounds: distant, possible pericardial effusion. • Pulse: bradycardia. • Neurologic: delayed relaxation phase of the DTRs, cerebellar ataxia, hearing impairment, poor memory or dementia, peripheral neuropathies with paresthesia. • Musculoskeletal: carpal tunnel syndrome, muscular stiffness, weakness.

ETIOLOGY PRIMARY HYPOTHYROIDISM (THYROID GLAND DYSFUNCTION): The cause of 90% of the cases of hypothyroidism • Hashimoto’s thyroiditis is the most common cause of hypothyroidism • Idiopathic myxedema (nongoitrous form of Hashimoto’s thyroiditis) • Previous treatment of hyperthyroidism (radioiodine therapy, subtotal thyroidectomy) • Subacute thyroiditis • Radiation therapy to the neck (usually for malignant disease) • Iodine deficiency or excess • Drugs (lithium, PAS, sulfonamides, phenylbutazone, amiodarone, thiourea) • Prolonged treatment with iodides SECONDARY HYPOTHYROIDISM: Pituitary dysfunction, postpartum necrosis, neoplasm, infiltrative disease causing deficiency of TSH TERTIARY HYPOTHYROIDISM: Hypothalamic disease (granuloma, neoplasm, or irradiation causing deficiency of TRH) TISSUE RESISTANCE TO THYROID HORMONE: Rare

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Depression • Dementia from other causes • Systemic disorders (e.g., nephrotic syndrome, CHF, amyloidosis)

LABORATORY TESTS • Increased TSH: TSH may be normal if patient has secondary or tertiary hypothyroidism, is receiving dopamine or corticosteroids, or the level is obtained following severe illness • Decreased free T4 • Other common laboratory abnormalities: hyperlipidemia, hyponatremia, and anemia • Increased antimicrosomal and antithyroglobulin antibody titers: useful when autoimmune thyroiditis is suspected as the cause of the hypothyroidism

TREATMENT NONPHARMACOLOGIC THERAPY Patients should be educated regarding hypothyroidism and its possible complications. Patients should also be in-

structed about the need for lifelong treatment and monitoring of their thyroid abnormality.

ACUTE GENERAL Rx Start replacement therapy with levothyroxine (Synthroid, Levothroid) 25 g/ day. The dose may be increased by 25 g/day every 6 to 8 wk, depending on the clinical response and serum TSH level. Elderly patients with coronary artery disease should be started with 12.5 g/day (higher doses may precipitate angina). The average maintenance dose of levothyroxine is 1.7 g/kg/day (100 to 150 g/day in adults). The elderly may require 1 g/kg/day.

CHRONIC Rx • Periodic monitoring of TSH level is an essential part of treatment. Patients should be evaluated initially with office visit and TSH levels every 6 to 8 wk until the patient is clinically euthyroid and the TSH level is normalized. The frequency of subsequent visits and TSH measurement can then be decreased to every 6 to 12 mo. • For monitoring therapy in patients with central hypothyroidism, measurement of serum free thyroxine (free T4 level) is appropriate and should be maintained in the upper half of the normal range.

REFERRAL Admission to the hospital ICU is recommended in all patients with myxedema coma.

PEARLS & CONSIDERATIONS COMMENTS Subclinical hypothyroidism occurs in as many as 15% of elderly patients and is characterized by an elevated serum TSH and a normal free T4 level. Treatment is individualized. Generally, replacement therapy is recommended for all patients with serum TSH 10 mU/L, patients with presence of goiter, patients with cognitive deficits, or obese patients. Some patients who report no symptoms prior to thyroid replacement feel more energetic when euthyroidism is restored. AUTHOR: FRED F. FERRI, M.D.

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Incontinence, Fecal

ETIOLOGY

TREATMENT

• Anatomic:  Sphincteric disruption, from vaginal deliveries, anorectal surgery, and trauma  Pelvic floor neuropathy with rectal prolapse, chronic straining at stool, and pelvic floor descent  Isolated degeneration of internal anal sphincter • Neurologic:  CNS damage or malfunction, including dementia  Diabetes mellitus, multiple sclerosis, and other specific entities • Functional:  Fecal impaction  Abnormal GI function with excessive stool volume and rapid gut transport  Laxative abuse  Transient fecal incontinence associated with diarrhea

NONPHARMACOLOGIC THERAPY

Fecal incontinence is the involuntary loss of stool through the anal canal.

ICD-9CM CODES 787.6 Fecal incontinence

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: • Extremely variable dependent on definition, site, and mode of collection of data • Average range: 0.004% to 18% • U.S. nursing homes report 47% DEMOGRAPHICS: • Over age 65: 30% • Females: 63% • Notable with advanced age, severe debility, and multiple comorbidities FREQUENCY (IN U.S. STUDY): • Daily: 2.7% of patients • Weekly: 4.5% • Monthly or less: 7.1% RISK FACTORS: • Constipation • Age 80 • Urinary incontinence • Impaired mobility • Dementia • Neurologic disease

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Uncontrolled loss of fecal matter or staining of underclothing • Often unreported to physicians, or reported as pruritus, diarrhea, or urgency • May cause complications, such as pressure sores and urinary infections, and may lead to isolation, avoidance of normal activities, and depression

DIAGNOSIS

• TSH • Electrolytes

IMAGING STUDIES • Abdominal x-ray

• Dietary changes: avoid food stimulants (e.g., lactose), add fiber • Habit training • Barrier creams to protect skin • Biofeedback by trained personnel

ACUTE GENERAL Rx • Antidiarrheal meds: loperamide, diphenoxylate, and bile-acid binders • If diarrhea is present, identify its etiology and treat it

REFERRAL • Gastroenterologist • Colorectal surgeon  Sphincteroplasty  Diversion of fecal stream (ostomy)

PEARLS & CONSIDERATIONS COMMENTS

DIFFERENTIAL DIAGNOSIS • Mainly requires establishing applicable etiology by history and physical exam • Uncontrolled diarrhea from diseases of the gut • Medication abuse, primarily laxatives

WORKUP • Colonoscopy for masses and inflammatory conditions when pathology suspected • Anorectal physiology testing: imaging, manometry, and EMG

• Severely annoying problem requiring proper protection and skin care. • Most cases are reversible, controllable, and manageable by generalist. • Complex and anatomic distortions will require specialist intervention. • Dementia and other neurologic problems may prevent cure.

SUGGESTED READINGS Madoff RD et al: Faecal incontinence in adults, Lancet 364:621, 2004. AUTHOR: E. GORDON MARGOLIN, M.D.

DISEASES AND DISORDERS

Uncontrollable loss of stool

DEFINITION

Section I

LABORATORY TESTS

SYNONYMS

• Inspection of anal area for deformed perineal body, scar formation, skin breakdown, gaping of anus • Digital rectal exam to evaluate anal tone and to identify fecal impaction • Evaluation of perineal sensation and anal wink reflex

BASIC INFORMATION

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Incontinence, Urinary BASIC INFORMATION



DEFINITION Urinary incontinence is defined as the uncontrolled loss of urine, generally in an undesirable place, creating social and hygienic problems. Recently, the definition has been broadened to encompass the related problem of the overactive bladder even when there is no accompanying loss of urine.



SYNONYMS Uncontrolled loss of urine Bladder accidents Overactive bladder ICD-9CM CODES 788.30 Urinary incontinence, including urge and functional 788.33 Mixed 625.6 Stress (females) 788.32 Stress (males) 788.39 Other 596.51 Overactive bladder

EPIDEMIOLOGY & DEMOGRAPHICS • Can present at any age. • Prevalence increases with advancing age. • More common in women than in men. • Estimated prevalence in elders: 30% in community dwellers, 50% in hospitalized individuals, and 70% or more in nursing home residents. • Annual costs in U.S. are estimated at $30 billion reflecting labor, laundry, institutional costs, products, and complicating events.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Types of incontinence—defined by duration:  Acute or transient (less than 2 months’ duration): may be due to, or associated with, delirium, infection, atrophic urethritis and vaginitis, pharmaceuticals, psychologic issues, excessive urine production, restricted mobility, and stool impaction (DIAPPERS).  Chronic or established (more than 2 months’ duration): described as follows. • Types of established incontinence— defined by manifestations:  Stress: incompetence of sphincteric mechanism, allowing spurts of urine when there are increases in intraabdominal pressure (such as coughing, lifting, etc.). Constant leakage can occur with totally incompetent sphincter (continuous incontinence).





Urge: overactivity of detrusor muscle, creating sudden losses of variable amounts of urine both with and without warning. Overactive bladder syndrome is the same mechanism with severe urgency but with capability to toilet quickly enough to prevent urinary losses. Overflow: sudden and uncontrolled emptying of an overdistended bladder, due to either obstruction to outflow or paralysis of the musculature of the detrusor. Symptoms may mimic those of Urge. Functional: despite normal lower urinary system, there is failure to toilet appropriately because of cognitive, physical, or environmental constraints. Mixed: combinations of any of the previous four types—especially Stress, Urge, and Functional—are common in the elderly.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Incontinence is frequently not reported to, or identified by, the physician. • Careful history taking can often determine the clinical type(s) of incontinence, but not necessarily the cause. It is essential to evaluate all medications being taken. • Abdominal, pelvic, and rectal examinations help identify enlarged bladder, anatomic distortions of pelvic organs, enlargement of prostate, impaction of stool. • Neurologic exam describes cerebral or spinal cord disease, peripheral neuropathy, gait limitations, and dexterity. • Levels of cognition, awareness, and motivation determine functional issues.

• Neurologic diseases, involving either the spinal cord or the brain • Infections and stones of the bladder and kidneys • Trauma to the bladder and its outlet, mechanical or radiation induced • Hormonal changes • Selected medications, such as -agonists, anticholinergics, sedatives • Diabetes mellitus and its consequences

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

Urinary tract infection Rectal mass or impaction Volume overload Atrophic vaginitis Benign prostatic hypertrophy Spinal cord injury Diabetes mellitus

WORKUP • Careful history and focused physical exam are the mainstay of evaluation. • Fluid intake and presence of edema are important considerations. • Voiding diary. • Urodynamic testing is not routinely indicated as first line.

LABORATORY TESTS • Urinalysis and urine culture and sensitivity • Renal function tests

IMAGING STUDIES • Bladder ultrasound. • Measurement of postvoid residual may be essential to identify Overflow and to distinguish it from Urge.

TREATMENT

ETIOLOGY

ACUTE GENERAL Rx

• Partly related to aging changes in physiology and anatomy of the lower urinary tract  Shrinkage of bladder  Increased number of uncontrolled detrusor contractions  Increase in postvoid volume and a decrease in bladder contractility  Loss of hormonal support of genitourinary tissues  Impaired responses to sensory stimuli  Frailty  Alteration of diurnal rhythms resulting in larger urinary flows at night • Changes of supporting tissues, often related to damage from childbirth • Enlargement of prostate in men

STRESS: • Pharmaceutical  -agonists (pseudoephedrine)  Local estrogen creams • Behavioral—see treatment for Urge • Products  Pessaries may benefit women with vaginal or uterine prolapse  Small pads for small losses  Briefs • Surgical  Needs as defined by surgical specialists include prostatectomy, pelvic repair, etc.

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Incontinence, Urinary 

DISPOSITION PROGNOSIS • In the more frail and dependent elderly individual, success rates are limited by virtue of noncompliance, complications and side effects of medicationss. • Use of products to contain urine when other measures fail can be most useful in management, in reduction of embarrassment, and to provide comfort and sense of security for the troubled patient.

REFERRAL Gynecologic, urologic, and urodynamic studies are required only with refractory or uncertain cases, when associated with hematuria or uncontrolled infections, where anatomic distortions need interventions, or when symptoms are atypical.

PEARLS & CONSIDERATIONS COMMENTS • A careful history should precede any further workup, including a diary maintained by the patient or caregiver. • Usual workup includes focused physical examination and limited laboratory measurement. • Behavioral therapy is preferred, if effective. • Current medications are fraught with side effects and must be used cautiously. New medications are in study. • Advising regarding products and activities, as well as supporting the patient, is an ongoing process.

SUGGESTED READINGS Holroyd-Leduc JM, Straus SE: Management of urinary incontinence in women, JAMA 291:996, 2004. Ouslander JG: Management of overactive bladder, N Engl J Med 350:786, 2004. Scientific Committee of the First International Consultation on Incontinence: Assessment and treatment of urinary incontinence, Lancet 355(9221):2153, 2000. Weiss BD: Diagnostic evaluation of urinary incontinence in geriatric patients, Am Fam Physician 57:2675, 1998. AUTHOR: E. GORDON MARGOLIN, M.D.

DISEASES AND DISORDERS

See Benign Prostatic Hyperplasia (BPH) in Section I FUNCTIONAL: • Dependent on cause  May require redirection  Frequent toileting  Assistive devices for mobility enhancement  Proximal placement of commode MIXED: • Best to treat each type separately, starting with the type causing the greatest nuisance.

Section I

URGE: • Pharmaceutical—anticholinergic drugs  Tolterodine  Oxybutynin  Darifenacin  Caution: systemic complications of these drugs and urination retention in BPH  Overactive bladder syndrome is also managed with these drugs • Behavioral  Bladder training (i.e., timed voiding and control)  Pelvic muscle exercises (Kegel) • Products  Large variety available to accommodate particular needs • Surgical  Experimental use of excisional techniques  Placement of “pacemaker-type” devices OVERFLOW: • Neurogenic causes may be helped with parasympathetic agonists (bethanechol). • Repeated catheterizations to empty the bladder may be necessary. • Obstructive causes are mechanical, requiring surgical intervention.  Especially after trials with 1-blockers used to relax the sphincter or finasteride to shrink the size of the prostate

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Influenza BASIC INFORMATION DEFINITION Influenza is an acute febrile illness caused by infection with influenza type A or B virus.

SYNONYMS Flu ICD-9CM CODES 487.1 Influenza

rapid diagnostic method in the setting of acute illness. • Specimens are placed into virus transport medium and processed by a reference laboratory. • For serologic diagnosis: 1. Paired serum specimens, acute and convalescent, the latter obtained 10 to 20 days later 2. Fourfold rises or falls in the titer of antibodies (various techniques) considered diagnostic of recent infection

EPIDEMIOLOGY & DEMOGRAPHICS

LABORATORY TESTS

INCIDENCE (IN U.S.): Annual incidence of influenza-related deaths is approximately 20,000 deaths/yr PREDOMINANT SEX: Male  female PEAK INCIDENCE: Winter outbreaks lasting 5 to 6 wk

IMAGING STUDIES

PHYSICAL FINDINGS & CLINICAL PRESENTATION • “Classic flu” is characterized by abrupt onset of fever, headache, myalgias, anorexia, and malaise after a 1- to 2day incubation period. • Clinical syndromes are similar to those produced by other respiratory viruses, including pharyngitis, common colds, tracheobronchitis, bronchiolitis, croup. • Respiratory symptoms such as cough, sore throat, and nasal discharge are usually present at the onset of illness, but systemic symptoms predominate. • Elderly patients may experience fever, weakness, and confusion without any respiratory complaints. • Acute deterioration to status asthmaticus may occur in patients with asthma. • Influenza pneumonia: rapidly progressive cough, dyspnea, and cyanosis may occur after typical flu onset.

ETIOLOGY • Variation in the surface antigens of the influenza virus, hemagglutinin (HA) and neuraminidase (NA), leading to infection with variants to which resistance is inadequate in the population at risk • Transmitted by small-particle aerosols and deposited on the respiratory tract epithelium

DIAGNOSIS DIFFERENTIAL DIAGNOSIS

Septic syndrome presentation: CBC, ABG analysis, blood cultures • Chest x-ray examination to demonstrate findings of viral pneumonia: peribronchial and patchy interstitial infiltrates in multiple lobes with atelectasis • Possible progression to diffuse interstitial pneumonitis

TREATMENT NONPHARMACOLOGIC THERAPY • Bed rest • Hydration

ACUTE GENERAL Rx • Supportive care: antipyretics • Antibiotics if bacterial pneumonia is proven or suspected • Amantadine (100 mg PO once daily in patients 65 yr) and rimantadine (same dose schedule as amantadine) 1. Further dose adjustments needed with renal insufficiency 2. Fewer CNS side effects with rimantadine • Neuraminidase inhibitors block release of virions from infected cells, resulting in shortened duration of symptoms and decrease in complications; effective against both influenza A and B 1. Zanamivir, administered via inhaler, 10 mg bid 2. Oseltamivir, administered orally • Placebo-controlled studies have suggested that antiviral therapy with any of the above mentioned agents must be initiated within 1 to 2 days of the onset of symptoms and reduces the duration of illness by approximately 1 day

• Adenovirus, parainfluenza virus infection • Secondary bacterial pneumonia or mixed bacterial-viral pneumonia

DISPOSITION

WORKUP

REFERRAL

• Virus isolation from nasal or throat swab or sputum specimens is the most

Patients are hospitalized if signs of pneumonia are present. Infectious disease and/or pulmonary consultation when influenza pneumonia is suspected

PEARLS & CONSIDERATIONS COMMENTS • Prevention of influenza in patients at high risk is an important goal of primary care. • Vaccines reduce the risk of infection and the severity of illness. 1. Antigenic composition of the vaccine is updated annually. 2. Vaccination should be given at the start of the flu season (October) for the following groups: a. Adults 65 yr b. Adults with chronic cardiac or pulmonary disease, including asthma c. Adults with illness requiring frequent follow-up (e.g., hemoglobinopathies, diabetes mellitus) d. Immunocompromised patients e. Household contacts of persons in the previous groups f. Health-care workers 3. Only contraindication to vaccination is hypersensitivity to hen’s eggs. 4. Special efforts should be made to vaccinate high-risk patients 65 yr, only 10% to 15% of whom are vaccinated each year. • Chemoprophylaxis: 1. Amantadine and rimantadine approved for prophylaxis against influenza A; they are ineffective against influenza B 2. Consider: a. For high-risk patients in whom vaccination is contraindicated b. When the available vaccine is known not to include the circulating strain c. To provide added protection to immunosuppressed patients likely to have a diminished response to vaccination d. In the setting of an outbreak, when immediate protection of unvaccinated or recently vaccinated patients is desired (e.g., in a nursing home) 3. Give for 2 wk in the case of late vaccination and for the duration of the flu season in all other patients

SUGGESTED READINGS Colgan R et al: Antiviral drugs in the immunocompetent host: part II. Treatment of influenza and respiratory syncytial virus infections, Am Fam Physician 67(4):763, 2003. Montalto NJ: An office-based approach to influenza: clinical diagnosis and laboratory testing, Am Fam Physician 67(1):111, 2003. AUTHOR: CLAUDIA L. DADE, M.D.

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Insomnia BASIC INFORMATION

SYNONYMS Sleeplessness, sleep disorder, sleep disturbance, dyssomnia. The terms sleep disorder, sleep disturbance, and dyssomnia are generic and can refer to disorders of wakefulness (hypersomnia) or sleep-related behavior disorders (parasomnias). ICD-9CM CODES 780.52 Insomnia 780.51 Insomnia with sleep apnea 307.41 Insomnia nonorganic origin 307.42 Insomnia persistent (primary) 307.41 Insomnia transient 307.49 Subjective complaint DSM IV-TR Codes: 307.42 Primary insomnia 307.45 Circadian rhythm disorders 780.52 Insomnia due to a general medical condition 291.89 Substance-induced insomnia due to alcohol 292.89 Substance-induced insomnia due to other (i.e., caffeine, drug)

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 30%-45% of adults experience insomnia per year. PREVALENCE (IN U.S.): 1%-15% of all adults develop persistent insomnia, 25% of older adults. PREDOMINANT SEX: More common in women. PREDOMINANT AGE: Transient insomnia is common at any age, persistent insomnia is more common in those 60 yrs. Older adults usually have more sleep maintenance difficulty. GENETICS: Both idiopathic primary insomnia and chronobiologic forms of insomnia run in families and may be genetically determined.

CLINICAL PRESENTATION • Complain of difficulty falling asleep, difficulty staying asleep, early morning awakening, restless or nonrestorative sleep, or difficulty sleeping at desired times. • May or may not complain of daytime sleepiness or fatigue.

ETIOLOGY • Transient insomnia: 1. Stress 2. Illness 3. Travel 4. Environmental disruptions (noise, heat, cold, poor bedding, unfamiliar surroundings, etc.) • Persistent insomnia: 1. Mood disorders (depression, hypomania/mania) 2. Primary or psychophysiologic (with or without poor sleep hygiene) 3. Sleep-related breathing disorders (e.g., obstructive apnea) 4. Chronobiologic (a.k.a. circadian rhythm) disorder (delayed sleep phase, advanced sleep phase, shift work, free-running rhythm secondary to blindness) 5. Drug and alcohol abuse 6. Restless legs and periodic leg movements 7. Neurodegenerative (Alzheimer’s disease, Parkinson’s disease, etc.) 8. Medical (pain, GERD, nocturia, orthopnea, medications, etc.)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Primary or psychophysiologic insomnia is diagnosed when other etiologies (see above) are ruled out.

WORKUP • History (with bed partner interview, if possible) • Sleep diary for 2 weeks to document severity, frequency, daytime function, and distress (sample sleep diary can be downloaded from the National Sleep Foundation website: http://www. sleepfoundation.org)

LABORATORY TESTS • Evaluate for anemia, uremia (for restless legs), thyroid function (if other signs present) • Polysomnography (in home or in sleep lab) for symptoms suggesting something other than primary insomnia: daytime sleepiness (obstructive sleep apnea, narcolepsy), nonrestorative sleep (periodic leg movements), or sleep behavior suggesting parasomnia (somnambulism, REM sleep behavior)

IMAGING STUDIES • Not generally helpful for insomnia • Brain CT or MRI for severe daytime sleepiness of acute onset

TREATMENT NONPHARMACOLOGIC THERAPY • Sleep hygiene measures (see Box 1-3). • Cognitive-behavioral therapy (CBT) to address anxiety and insomnia-perpetuating behaviors. • Bright light exposure timed to correct a circadian phase disturbance can be helpful for insomnia secondary to delayed or advanced sleep phase, jet lag, or shift work.

ACUTE GENERAL Rx • Benzodiazepine sedative-hypnotics (e.g., temazepam 7.5-30 mg, triazolam 0.125-0.25 mg) should generally be avoided in the geriatric population. 1. In critical care: lorazepam 0.25-0.5 mg PO, SL, or IV as needed for sleep.

Sleep Habits (Sleep Hygiene Measures) That May Improve Insomnia BOX 1-3

1. 2. 3. 4. 5. 6. 7. 8. 9. 10.

Reduce caffeine, alcohol, or tobacco late in the day or evening. Avoid heavy meals at night. Increase daytime activity. Increase daytime exposure to natural light. Take warm bath as part of bedtime ritual. Restrict bed to sleep and sex. Get out of bed if not asleep after 30 minutes and return when drowsy. Repeat above if awakened during the night. Maintain regular sleep and wake times. Go to bed with calm mind; resolve arguments or deal with problems earlier in day.

DISEASES AND DISORDERS

Insomnia is a disturbance of initiating or maintaining sleep. Restless, nonrestorative sleep may also be described as insomnia. The disturbance may be subjective without daytime sequelae but still a cause of distress, or may be objectively measurable with poor sleep efficiency and daytime consequences of sleepiness and functional impairment.

• Validated sleep-quality rating scale (optional) 1. Pittsburgh Sleep Quality Index or similar questionnaire 2. Epworth Sleepiness Scale (see Daytime Sleepiness Test at http://www. sleepfoundation.org)

Section I

DEFINITION

• Symptoms may be acute and self-limited, chronic but intermittent, or chronic and frequent.

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Insomnia • Benzodiazepine receptor agonists (e.g., zolpidem 5-10 mg for sleep-onset and maintenance insomnia, zaleplon 5-10 mg for sleep-onset insomnia) eszopiclone 1 mg qhs for sleep initiation or 2 mg qhs for sleep maintenance. • Avoid antihistamines. • Optimize treatment of medical symptoms, especially pain.

CHRONIC Rx • Ramelteon 8 mg qhs is a melatonin agonist and can be used on a longterm basis. • Eszopiclone and zolpidem CR (6.25 or 12.5 mg tablets) can also be used longterm. • Some evidence that benzodiazepines and benzodiazepine receptor agonists can be used for chronic insomnia on either intermittent or nightly use with moderate risk of tolerance and dependence but low risk of addiction. • Sedating antidepressants (e.g., trazodone 25-150 mg, mirtazapine 7.5-30 mg) in widespread use but limited data on safety and efficacy for insomnia. Treatments of choice for comorbid depression or anxiety. Amitriptyline should be avoided if possible in older adults. • Sedating antipsychotics (e.g., quetiapine 25-200 mg, olanzapine 2.5-10 mg) for severe mood or psychotic disorders associated with insomnia.

COMPLEMENTARY & ALTERNATIVE MEDICINE Melatonin is the only substance that has been studied in larger controlled trials. It may shorten sleep-onset latency in some

individuals. Melatonin can be very effective for insomnia due to circadian rhythm disturbances if scheduled to correct the underlying circadian phase disturbance.

DISPOSITION • Transient insomnia: usually self-limited, but may require follow-up if stress- or illness-related because of risk of depression or persistent insomnia. • Persistent insomnia: patients have a chronic and recurrent disorder and will need periodic follow-up to reinforce good sleep hygiene measures and to reassess need for pharmacologic and nonpharmacologic therapies.

REFERRAL • Excessive daytime sleepiness not obviously due to insomnia (e.g., narcolepsy, sleep-related breathing disorder, etc.) • Nighttime behavior suggestive of a parasomnia (e.g., somnambulism, REM behavior disorder, etc.) • Severe insomnia not responsive to basic interventions

PEARLS & CONSIDERATIONS COMMENTS Treatment of insomnia should focus on reducing daytime sleepiness and improving daytime function, rather than trying to achieve the elusive goal of uninterrupted nighttime sleep.

PREVENTION Not much is known about prevention of insomnia. Effective treatment of transient insomnia may reduce the risk of developing persistent insomnia.

PATIENT/FAMILY EDUCATION The National Sleep Foundation (http://www.sleepfoundation.org) is a comprehensive resource for health care providers and patients.

SUGGESTED READINGS Jacobs GD et al: Cognitive behavior therapy and pharmacotherapy for insomnia: a randomized controlled trial and direct comparison, Arch Int Med 164(17):1888, 2004. Krystal AD: The changing perspective on chronic insomnia management, J Clin Psychiatry 65(Suppl 8):20, 2004. Ringdahl EN, Pereira SL, Delzell JE: Treatment of primary insomnia, J Am Board Fam Pract Behavioural Medicine in Primary Care: A Practical Guide, ed 2, New York, 2003, Lange Medical Books/McGraw Hill. Smith MT et al: Comparative meta-analysis of pharmacotherapy and behaviour therapy for persistent insomnia, Am J Psychiatry 159:5, 2002. AUTHOR: CLIFFORD MILO SINGER, M.D.

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Irritable Bowel Syndrome BASIC INFORMATION Irritable bowel syndrome (IBS) is a chronic functional disorder manifested by alteration in bowel habits and recurrent abdominal pain and bloating. Irritable colon Spastic colon IBS ICD-9CM CODES 564.1 Irritable bowel syndrome

EPIDEMIOLOGY & DEMOGRAPHICS • IBS occurs in 20% of population of industrialized countries and is responsible for 50% of GI referrals. Worldwide adult prevalence is 12%. • Female:male ratio is 2:1. • Nearly 50% of patients have psychiatric abnormalities, with anxiety disorders being most common.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • The clinical presentation of IBS consists of abdominal pain and abnormalities of defecation, which may include loose stools usually after meals and in the morning, alternating with episodes of constipation. • Physical examination is generally normal. • Nonspecific abdominal tenderness and distention may be present.

ETIOLOGY • Unknown • Associated pathophysiology includes altered GI motility and increased gut sensitivity • Risk factors: anxiety, depression, personality disorders, history of childhood sexual abuse, and domestic abuse in women

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • •

IBD Diverticulitis Colon malignancy PUD Biliary liver disease Chronic pancreatitis

WORKUP Diagnostic workup is aimed primarily at excluding the conditions listed in the differential diagnoses. It is important to identify “red flags” of other diseases, such as weight loss, rectal bleeding, onset in patients 50 years of age, fever, nocturnal pain, family history of malignancy.

LABORATORY TESTS • Blood work is generally normal. The presence of anemia should alert to the possibility of a colonic malignancy or IBD. • Testing of stool for ova and parasites and for celiac disease should be considered in patients with chronic diarrhea.

IMAGING STUDIES • Small bowel series and barium enema are normal and not necessary for diagnosis. • Lower endoscopy is generally normal except for the presence of some spasms.

TREATMENT NONPHARMACOLOGIC THERAPY • The patient should be encouraged to maintain a high-fiber diet and to eliminate foods that aggravate symptoms. Avoidance of dietary caffeine and dietary excesses is also helpful. • Behavioral therapy is also recommended because psychosocial stressors are important triggers of IBS. • Importance of regular exercise and adequate fluid intake should be stressed.

GENERAL Rx • The mainstay of treatment of IBS is high-fiber diet. Because symptoms are chronic, the use of laxatives should be avoided. • Fiber supplementation with psyllium 1 to 2 tablespoons bid or calcium polycarbophil (FiberCon) 2 tablets one to four times daily followed by 8 oz of water may be necessary in some patients. • Patients should be instructed that there might be some increased bloating on initiation of fiber supplementation, which should resolve within 2 to 3 wk. It is important that patients take these fiber products on a regular basis and not only prn. Slow titration may help. • Antispasmodics-anticholinergics may be useful in refractory cases (e.g., di-

DISPOSITION Greater than 60% of patients respond successfully to treatment over the initial 12 mo; however, IBS is a chronic relapsing condition and requires prolonged therapy.

REFERRAL GI referral is recommended in patients with rectal bleeding, fever, nocturnal diarrhea, anemia, weight loss, or onset of symptoms after age 40 yr.

PEARLS & CONSIDERATIONS COMMENTS • Patients should be educated regarding maintenance of high-fiber diet and elimination of stressors, which can precipitate attacks of IBS. They should be reassured that their condition cannot lead to cancer. • The modified Rome criteria define IBS as: A. The presence of 12 wk of continuous or recurrent abdominal pain or discomfort that cannot be explained by structural or biochemical abnormalities and B. The presence of at least two of the following three features: 1. Pain is relieved with defecation 2. Its onset is associated with a change in the frequency of bowel movement 3. Its onset is associated with a change in the form of the stool.

SUGGESTED READINGS Mertz HR: Irritable bowel syndrome, N Engl J Med 349:22, 2003. Viera AJ et al: Management of irritable bowel syndrome, Am Fam Physician 66:1867, 2002. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

SYNONYMS

cyclomine [Bentyl] 10 to 20 mg up to three times daily). • Patients who appear anxious can benefit from use of sedatives and anticholinergics such as chlordiazepoxideclidinium (Librax) or SSRIs. • Loperamide is effective for diarrhea. • Tegaserod (Zelnorm), a 5-HT4 receptor partial agonist, increases GI motility and can be used to relieve symptoms in patients whose predominant symptom is constipation. Usual dose is 2 to 6 mg PO bid before meals. Tegaserod is contraindicated in patients with severe renal insufficiency, moderate to severe hepatic impairment, intestinal adhesions, or a history of bowel obstruction.

Section I

DEFINITION

The criteria for diagnosis of IBS are: more than 3 months of symptoms including abdominal pain that is relieved by a bowel movement, or pain accompanied by a change in bowel pattern, and abnormality in bowel movement 25% of the time, characterized by two of the following features: • Abdominal distention • Abnormal consistency • Abnormal defecation (e.g., straining, sense of incomplete evacuation) • Abnormal frequency • Mucus with bowel movement

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BASIC INFORMATION DEFINITION Cancer of the larynx, including the vocal cords (glottis), supraglottis, and subglottis.

SYNONYMS Laryngeal cancer Head and neck cancer (subsite); other sites include oral cavity, pharynx, perinasal sinus, and salivary glands ICD-9CM CODES 231.0 Carcinoma of larynx

EPIDEMIOLOGY & DEMOGRAPHICS • 12,000 new cases per year in the U.S. • 80% male predominance (current, with past and projected increase in female rates as a result of changing smoking habits) • Peak incidence in sixth decade

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Glottis Early diagnosis possible because of voice change (hoarseness). Any voice change of more than 2 wk duration should prompt a laryngeal examination. • Supraglottis 1. No early symptom 2. Cervical lymphadenopathy 3. Neck pain or ear pain 4. Discomfort during swallowing 5. Odynophagia 6. Later: hoarseness, dysphagia, airway obstruction • Subglottis Even more subtle than supraglottic lesion; the same signs occur, only later in the course

ETIOLOGY • • • • • • • • • •

Smoking (cigarette, cigar, or pipe) Alcohol intake/abuse Diet and nutritional deficiencies Gastroesophageal reflux Voice abuse Chronic laryngitis Exposure to wood dust Asbestosis Exposure to radiation Possible role of human papilloma virus

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • •

Laryngitis Allergic and nonallergic rhinosinusitis Gastroesophageal reflux Voice abuse leading to hoarseness Laryngeal papilloma

• Vocal cord paralysis secondary to a neurologic condition or secondary to entrapment of the recurrent laryngeal nerve caused by mediastinal compression • Tracheomalacia STAGING: Supraglottic

T1 Tumor limited to one subsite with normal cord mobility T2 Tumor invades mucosa of more than one subsite (e.g., base of tongue, vallecula, pyriform sinus) without fixation of larynx T3 Tumor limited to larynx with vocal cord fixation or invasion of postcricoid area or preepiglottis T4 Tumor invades thyroid cartilage or extends into soft tissue of the neck, thyroid, or esophagus Glottic

T1 Tumor limited to vocal cord with normal mobility T1a Tumor limited to one vocal cord T1b Tumor involves both vocal cords T2 Tumor extends to supra- or subglottis or impairs cord mobility T3 Tumor limited to larynx with cord fixation T4 Tumor invades through cartilage or other tissues beyond larynx Stage grouping

Stage I: T1, N0, M0 Stage II: T2, N0, M0 Stage III: T3, N0, M0 T1, T2, T3, N1, M0 Stage IV: T4, N0, N1, M0 Any T, N2, N3, M0 Any T, any N or M 0

WORKUP • Laboratory: none • Endoscopic laryngeal inspection • After (and only after) diagnosis of the malignancy, imaging with CT or MRI should be undertaken to stage the disease HISTOLOGIC CLASSIFICATION: Epithelial cancers • Squamous cell carcinoma in situ • Superficially invasive cancer • Verrucous carcinoma • Pseudosarcoma • Anaplastic cancer • Transitional cell carcinoma • Lymphoepithelial cancer • Adenocarcinoma • Neuroendocrine tumors, including small cell and carcinoid Sarcomas Metastatic malignancies

TREATMENT ACUTE GENERAL Rx • Early Stage (T or T2): two options 1. Conservative surgery (partial laryngectomy) with neck dissection 2. Primary radiation

• Intermediate Stage: four options 1. Primary radiation alone 2. Supraglottic laryngectomy with neck dissection 3. Supraglottic laryngectomy with postoperative radiation 4. Chemotherapy with radiation • Advanced Stage Chemotherapy and radiation with total laryngectomy reserved for treatment failure Glottis • Carcinoma in situ 1. Microexcision 2. Laser vaporization 3. Radiation • Early Stage (T or T2): two options 1. Voice conservation surgery 2. Radiation • Intermediate Stage (T3) 1. Combined radiation and chemotherapy (cisplatin and 5FU) 2. Total laryngectomy for treatment failure • Advanced Stage (T4) 1. Combined radiation and chemotherapy 2. Total laryngectomy and neck dissection followed by postoperative radiation in unfavorable lesion or treatment failure Subglottis Total laryngectomy and approximate neck surgery to excise the tumor, followed by radiation Unresected Cancers • Induction chemotherapy and radiation followed by neck dissection in chemosensitive tumors, or by laryngectomy and neck dissection in chemoresistant tumors • If hypopharyngeal involvement exists: laryngopharyngectomy, neck dissection, and postoperative radiation

DISPOSITION Supraglottis 5-yr control • T1 95% to 100% • T2 80% to 90% • T3 65% to 85% • T4 40% to 55% Glottis 5-yr control • T1 95% to 100% • T2 50% to 85% • T3 35% to 85% • T4 20% to 65%

SUGGESTED READING Sessions RB, Harrison LB, Forastiere AA: Tumors of the larynx and hypopharynx. In Cancer: Principles and Practice of Oncology, ed 6, Philadelphia, 2001, Lippincott Williams & Wilkins. AUTHOR: TOM J. WACHTEL, M.D.

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Leukemia, Chronic Lymphocytic BASIC INFORMATION

SYNONYMS CLL ICD-9CM CODES 204.1 Leukemia, chronic lymphocytic

EPIDEMIOLOGY & DEMOGRAPHICS • Most frequent form of leukemia in Western countries (10,000 new cases/yr in the U.S.) • Generally occurs in middle-aged and elderly patients (median age of 65 yr) • Male:female ratio of 2:1

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Lymphadenopathy, splenomegaly, and hepatomegaly in the majority of patients • Variable clinical presentation according to stage of the disease • Abnormal CBC: many cases are diagnosed on the basis of laboratory results obtained after routine physical examination • Some patients come to medical attention because of weakness and fatigue (secondary to anemia) or lymphadenopathy

ETIOLOGY Unknown

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • •

Hairy cell leukemia Adult T cell lymphoma Prolymphocytic leukemia Viral infections Waldenström’s macroglobulinemia

WORKUP • Laboratory evaluation • Bone marrow aspirate • Chromosome analysis

LABORATORY TESTS • Proliferative lymphocytosis (15,000/dl) of well-differentiated lymphocytes is the hallmark of CLL. • There is monotonous replacement of the bone marrow by small lymphocytes (marrow contains 30% of welldifferentiated lymphocytes).

IMAGING STUDIES CT scan of abdomen to evaluate for hepatomegaly and splenomegaly

TREATMENT NONPHARMACOLOGIC THERAPY • Treatment goals are relief of symptoms and prolongation of life. • Observation is appropriate for patients in Rai Stage 0 or Binet Stage A.

ACUTE GENERAL Rx • Symptomatic patients in Rai Stage I and II or Binet Stage B: chlorambucil; local irradiation for isolated symptomatic lymphadenopathy and lymph nodes that interfere with vital organs

CHRONIC Rx Treatment of systemic complications: • Hypogammaglobulinemia is frequent in CLL and is the chief cause of infections. Immune globulin (250 mg/kg IV every 4 wk) may prevent infections but has no effect on survival. Infections should be treated with broad-spectrum antibiotics. Patients should be monitored for opportunistic infections. • Recombinant hematopoietic cofactors (e.g., granulocyte-macrophage colony– stimulating factor and granulocyte colony–stimulating factor) may be useful to overcome neutropenia related to treatment. • Erythropoietin may be useful to treat anemia that is unresponsive to other measures.

DISPOSITION The patient’s prognosis is directly related to the clinical stage (e.g., the average survival in patients in Rai Stage 0 or Binet Stage A is 120 mo, whereas for RAI Stage 4 or Binet Stage C it is approximately 30 mo). Overall 5-yr survival is 60%.

PEARLS & CONSIDERATIONS COMMENTS Long-term follow-up and frequency of follow-up are generally determined by the pace of the disease.

SUGGESTED READING Shanafelt TD, Call TG: Current approach to diagnosis and management of chronic lymphocytic leukemia, Mayo Clin Proc 79:388, 2004. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Chronic lymphocytic leukemia (CLL) is a lymphoproliferative disorder characterized by proliferation and accumulation of mature-appearing neoplastic lymphocytes.

• Fludarabine is an effective treatment for CLL that does not respond to initial treatment with chlorambucil. Recent reports indicate that when used as the initial treatment for CLL, fludarabine yields higher response rates and a longer duration of remission and progression-free survival than chlorambucil; overall survival, however, is not enhanced. • Rai Stages III and IV, Binet Stage C: chlorambucil chemotherapy with or without prednisone 1. Fludarabine, CAP (cyclophosphamide, Adriamycin, prednisone), or cyclophosphamide, doxorubicin, vincristine, and prednisone (miniCHOP) can be used in patients who respond poorly to chlorambucil. 2. Splenic irradiation can be used in selected patients with advanced disease.

Section I

DEFINITION

• Hypogammaglobulinemia and elevated LDH may be present at the time of diagnosis. • Anemia or thrombocytopenia, if present, indicates poor prognosis. • Trisomy-12 is the most common chromosomal abnormality, followed by 14 q, 13 q, and 11 q; these all indicate a poor prognosis. • New laboratory techniques (CD 38, fluorescence in situ hybridization [FISH]) can identify patients with earlystage CLL at higher risk of rapid disease progression. STAGING • Rai et al divided CLL into five clinical stages: Stage 0—Characterized by lymphocytosis only (15,000/mm3 on peripheral smear, bone marrow aspirate 40% lymphocytes). The coexistence of lymphocytosis and other factors increases the clinical stage. Stage 1—Lymphadenopathy Stage 2—Lymphadenopathy/hepatomegaly Stage 3—Anemia (Hgb 11 g/mm3) Stage 4—Thrombocytopenia (platelets 100,000/mm3) • Another well-known staging system developed by Binet divides chronic lymphocytic leukemia into three stages: Stage A—Hgb 10 g/dl, platelets 100,000/mm3, and fewer than three areas involved (the cervical, axillary, and inguinal lymph nodes [whether unilaterally or bilaterally]; the spleen; and the liver) Stage B—Hgb 10 g/dl, platelets 100,000/mm3, and three or more areas involved Stage C—Hgb 10 g/dl, low platelets (100,000/mm3), or both (independent of the areas involved)

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Lichen Sclerosus BASIC INFORMATION

ETIOLOGY

GENERAL Rx

DEFINITION

Unknown. There may be an autoimmune association and a genetic familial component.

Diagnosis is based on close examination of the lesions for the presence of ivorywhite atrophic lesions and typical location.

• Application of clobetasol propionate 0.05% topically bid for up to 4 wk is usually effective. Repeat courses of corticosteroids may be necessary because of the chronic nature of this disorder. Continual application of topical steroids may lead to atrophy of the vulva. • Use of topical testosterone (2%) has been found to be less effective than topical corticosteroids. • Lubricants (e.g., Nutraplus cream) are useful to soothe dry tissues. • Hydroxyzine 25 mg at hs is effective in decreasing nocturnal itching. • Use of intralesional steroids, etretinate, and surgical management are usually reserved for refractory cases.

LABORATORY TESTS

DISPOSITION

Chronic inflammatory condition of the skin usually affecting the vulva, perianal area, and groin ICD-9CM CODES 701.0 Lichen sclerosus

EPIDEMIOLOGY & DEMOGRAPHICS • Most common in postmenopausal women and men between ages 40 and 60 yr • More common in females

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Erythema may be the only initial sign. A characteristic finding is the presence of ivory-white atrophic lesions on the involved area. • Close inspection of the affected area will reveal the presence of white-tobrown follicular plugs on the surface (dells). • When the genitals are involved, the white parchmentlike skin assumes an hourglass configuration around the introital and perianal area (“keyhole” distribution, see Fig. 1-23). Inflammation, subepithelial hemorrhages, and chronic ulceration may develop. • Dyspareunia, genital bleeding, and anal bleeding are common.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Localized scleroderma (morphea) • Cutaneous discoid lupus erythematosus • Atrophic lichen planus • Psoriasis

WORKUP

Punch or deep shave biopsy can be used to confirm the diagnosis when in doubt.

IMAGING STUDIES Not indicated

TREATMENT

• The disease persists in approximately one third of patients. • Squamous cell carcinoma can develop within the lesions in 3% to 10% of older patients; therefore, periodic examination and biopsy of suspicious areas are indicated.

NONPHARMACOLOGIC THERAPY

PEARLS & CONSIDERATIONS

Attention to hygiene and elimination of irritants or excessive bathing with harsh soaps

COMMENTS • Lichen sclerosus of the vulva (kraurosis vulvae) usually occurs after menopause and is generally chronic. It can be painful and interfere with sexual activity. • Lichen sclerosus of the penis (balanitis xerotica obliterans) is seen more commonly in uncircumcised males. It affects the glans and prepuce and may lead to stricture if it encroaches into the urinary meatus. AUTHOR: FRED F. FERRI, M.D.

FIGURE 1-23 Lichen sclerosus. Perianal area is thinned and chalk white (keyhole distribution). (Courtesy Department of Dermatology, University of North Carolina at Chapel Hill. From Goldstein BG, Goldstein AO: Practical dermatology, ed 2, St Louis, 1997, Mosby.)

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Lumbar Disk Syndrome BASIC INFORMATION

ICD-9CM CODES 722.10 Lumbar disk displacement 724.02 Lumbar stenosis 344.60 Cauda equina syndrome 721.3 Lumbar spondylosis

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: • Variable • At least one episode in 80% of adults PREVALENT SEX: Approximately equal

PHYSICAL FINDINGS & CLINICAL PRESENTATION (TABLE 1-8) • Overlapping clinical syndromes that may result: 1. Mild herniation without nerve root compression 2. Herniation with nerve root compression 3. Cauda equina syndrome 4. Chronic degenerative disease with or without leg symptoms 5. Spinal stenosis • Low back pain, often worsened by activity or coughing and sneezing • Local lumbar or lumbosacral tenderness • Paresthesias, usually unilateral • Restricted low back motion • Increased pain on bending toward affected side • Weakness and reflex changes • Sensory examination usually not helpful • Lumbar stenosis that possibly produces symptoms (pseudoclaudication), which are often misinterpreted as being vascular

TABLE 1-8

L4 L5 S1 S2-4

ETIOLOGY

PHARMACOLOGIC THERAPY

Unknown

• • • •

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Soft-tissue strain/sprain • Tumor • Degenerative arthritis of hip • Insufficiency fracture of hip or pelvis Section II describes the differential diagnosis of common low back pain syndromes.

WORKUP In most cases, the diagnosis can be established on a clinical basis alone.

IMAGING STUDIES • Plain roentgenograms may be indicated within the first few weeks; they are usually normal in soft disk herniation, but with chronic degenerative disk disease, loss of height of the disk space and osteophyte formation can occur. • CT scanning and MRI may be indicated in patients whose symptoms do not resolve or when other spinal pathology may be suspected. • Electrodiagnostic studies may confirm the diagnosis or rule out peripheral nerve disorders.

TREATMENT NONPHARMACOLOGIC THERAPY • Short course (3 to 5 days) of bed rest for severe pain; prolonged rest for acute disk herniation with leg pain • Physical therapy for modalities plus a careful gradual exercise program • Lumbosacral corset brace during rehabilitation process in conjunction with exercise program

NSAIDs Muscle relaxants for sedative effect Analgesics Epidural steroid injection for leg symptoms in selected patients

DISPOSITION • Almost all lumbar disk syndromes improve with time. • Recurrent episodes usually respond to medical management. • Recovery from the rare paralytic event is often incomplete.

REFERRAL • For orthopedic or neurosurgical consultation for intractable pain or significant neurologic deficit • Emergency referral for cauda equina syndrome

PEARLS & CONSIDERATIONS COMMENTS • Surgery is more consistently helpful when leg pain (not back pain) predominates. • A clinical algorithm for evaluation of back pain is described in Section III.

SUGGESTED READINGS Biyani A, Andersson GB: Low back pain: Pathophysiology and management, J Am Acad Orthop Surg 12:106, 2004. Brodke DS, Ritter SM: Nonoperative management of low back pain and lumbar disc degeneration, J Bone Joint Surg 86A:1810, 2004. Buchner M, Schilotenwolf M: Cauda equina syndrome caused by intervertebral lumbar disc prolapse: mid-term results of 22 patients and literature review, Orthopedics 25:727, 2002. Butterman GR: Treatment of lumbar disc herniation: epidural steroid infection compared with discectomy: a prospective, randomized study, J Bone Joint Surg 86A:670, 2004.

Diagnosis of Lower Lumbar and Sacral Radiculopathy

Pain

Weakness (Selected Muscles)

Sensory Loss

Reflex Loss

Across thigh and medial leg to medial malleolus Posterior thigh and lateral calf, dorsum of foot Buttock and posterior thigh, calf, and lateral foot Posterior thigh, buttock, and genitalia

Quadriceps, thigh adductors, tibialis anterior Extensor digitorum brevis and longus, peronei Extensor digitorum brevis, peronei, gastrocnemius, soleus Gastrocnemius, soleus, abductor hallucis, abductor digiti quinti pedis, sphincter muscles

Medial leg

Knee

From Goldman L, Bennett JC (eds): Cecil textbook of medicine, ed 21, Philadelphia, 2000, WB Saunders.

Dorsum of foot Sole or lateral border of foot

Ankle

Buttocks, anal region, and genitalia

Bulbocavernosus, anal

DISEASES AND DISORDERS

Lumbar disk syndromes are diseases resulting from disk disorder, either herniation or degenerative change (spondylosis). Massive disk protrusion may rarely lead to paralysis in the lower extremity, a condition termed cauda equina syndrome. Gradual narrowing of the spinal canal (lumbar stenosis), usually from spondylosis, may also cause lower extremity symptoms.

• Percutaneous electrical nerve stimulation (PENS) may be beneficial in selected patients with chronic back pain

Section I

DEFINITION

• Positive straight leg raising test if nerve root compression is present • Also see Spinal Stenosis in Section I

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Lumbar Disk Syndrome Dreyfuss P et al: Sacroiliac joint pain, J Am Acad Orthop Surg 12:255, 2004. Kawaguchi Y et al: The association of lumbar disc disease with vitamin-D receptor gene polymorphism, J Bone Joint Surg 84(a):2022, 2002. Paassilta P et al: Identification of a novel common genetic risk factor for lumbar disc disease, JAMA 285:1843, 2001. Robinson LR: Role of neurophysiologic evaluation in diagnosis, J Am Acad Orthop Surg 8:190, 2000.

Silber JS et al: Advances in surgical management of lumbar degenerative disc disease, Orthopedics 25:767, 2002. Simotas AC: Non-operative treatment for lumbar spinal stenosis, Clin Orthop 384:153, 2001. Swenson R, Haldeman S: Spinal manipulation for low back pain, J Am Acad Orthop Surg 11:228, 2003. Tribus CB: Degenerative lumbar scoliosis: evaluation and management, J Am Acad Orthop Surg 11:174, 2003.

Wetzel FT, McNally TA: Treatment of chronic discogenic low back pain with intradiskal electrothermal therapy, J Am Acad Orthop Surg 11:6, 2003. Yoshihara K et al: Atrophy of the multifidus muscle in patients with lumbar disc herniation: histochemical and electromyographic study, Orthopedics 26:493, 2003. AUTHOR: LONNIE R. MERCIER, M.D.

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Lung Neoplasms, Primary BASIC INFORMATION

SYNONYMS Lung cancer ICD-9CM CODES 162.9 Malignant neoplasm of bronchus and lung, unspecified

EPIDEMIOLOGY & DEMOGRAPHICS • Lung cancer is responsible for 30% of cancer deaths in males and 25% of cancer deaths in females. • Tobacco smoking is implicated in 85% of cases; second-hand smoke is responsible for approximately 20% of cases. • There are 180,000 new cases of lung cancer yearly in the U.S., most occurring age 50 yr.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Weight loss, fatigue, fever, anorexia, dysphagia • Cough, hemoptysis, dyspnea, wheezing • Chest, shoulder, and bone pain

ETIOLOGY • Tobacco abuse • Environmental agents (e.g., radon) and industrial agents (e.g., ionizing radiation, asbestos, nickel, uranium, vinyl chloride, chromium, arsenic, coal dust)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • • •

Pneumonia TB Metastatic carcinoma to the lung Lung abscess Granulomatous disease Carcinoid tumor Mycobacterial and fungal diseases Sarcoidosis Viral pneumonitis Benign lesions that simulate thoracic malignancy: 1. Lobar atelectasis: pneumonia, TB, chronic inflammatory disease, allergic bronchopulmonary aspergillosis

WORKUP Workup generally includes chest x-ray, CT scan of chest, PET scan, and tissue biopsy.

LABORATORY TESTS Obtain tissue diagnosis. Various modalities are available: • Biopsy of any suspicious lymph nodes (e.g., supraclavicular node) • Flexible fiberoptic bronchoscopy: brush and biopsy specimens are obtained from any visualized endobronchial lesions • Transbronchial needle aspiration: done via a special needle passed through the bronchoscope; this technique is useful to sample mediastinal masses or paratracheal lymph nodes • Transthoracic fine-needle aspiration biopsy with fluoroscopic or CT scan guidance to evaluate peripheral pulmonary nodules • Mediastinoscopy and anteromedial sternotomy in suspected tumor involvement of the mediastinum • Pleural biopsy in patients with pleural effusion • Thoracentesis of pleural effusion and cytologic evaluation of the obtained fluid: may confirm diagnosis

IMAGING STUDIES • Chest x-ray: The radiographic presentation often varies with the cell type. Pleural effusion, lobar atelectasis, and mediastinal adenopathy can accompany any cell types. • CT scan of chest: to evaluate mediastinal and pleural extension of suspected lung neoplasms. • Positron emission tomography (PET) with 18F-fluorodeoxyglucose (18 FDGPET), a metabolic marker of malignant tissue, is superior to CT scan in detecting mediastinal and distant metastases in non–small cell lung cancer. It is useful for preoperative staging of non–small cell lung cancer. STAGING • Following confirmation of diagnosis, patients should undergo staging: 1. The international staging system is the most widely accepted staging

DISEASES AND DISORDERS

A primary lung neoplasm is a malignancy arising from lung tissue. The World Health Organization distinguishes 12 types of pulmonary neoplasms. Among them, the major types are squamous cell carcinoma, adenocarcinoma, small cell carcinoma, and large cell carcinoma. However, the crucial difference in the diagnosis of lung cancer is between small cell and non–small cell types, because the therapeutic approach is different. Selective characteristics of lung carcinomas: ADENOCARCINOMA: Represents 35% of lung carcinomas; frequently located in mid lung and periphery; initial metastases are to lymphatics, frequently associated with peripheral scars SQUAMOUS CELL (EPIDERMOID): 20% to 30% of lung cancers; central location; metastasis by local invasion; frequent cavitation and obstructive phenomena SMALL CELL (OAT CELL): 20% of lung carcinomas; central location; metastasis through lymphatics; associated with lesion of the short arm of chromosome 3; high cavitation rate LARGE CELL: 15% to 20% of lung carcinomas; frequently located in the periphery; metastasis to CNS and mediastinum; rapid growth rate with early metastasis BRONCHOALVEOLAR: 5% of lung carcinomas; frequently located in the periphery; may be bilateral; initial metastasis through lymphatic, hematogenous, and local invasion; no correlation with cigarette smoking; cavitation rare

2. Multiple pulmonary nodules: septic emboli, Wegener’s granulomatosis, sarcoidosis, rheumatoid nodules, fungal disease, multiple pulmonary AV fistulas 3. Mediastinal adenopathy: sarcoidosis, lymphoma, primary TB, fungal disease, silicosis, pneumoconiosis, drug-induced (e.g., phenytoin, trimethadione) 4. Pleural effusion: CHF, pneumonia with parapneumonic effusion, TB, viral pneumonitis, ascites, pancreatitis, collagen-vascular disease

Section I

DEFINITION

• Paraneoplastic syndromes: 1. Eaton-Lambert syndrome: myopathy involving proximal muscle groups 2. Endocrine manifestations: hypercalcemia, ectopic ACTH, SIADH 3. Neurologic: subacute cerebellar degeneration, peripheral neuropathy, cortical degeneration 4. Musculoskeletal: polymyositis, clubbing, hypertrophic pulmonary osteoarthropathy 5. Hematologic or vascular: migratory thrombophlebitis, marantic thrombosis, anemia, thrombocytosis, or thrombocytopenia 6. Cutaneous: acanthosis nigricans, dermatomyositis • Pleural effusion (10% of patients), recurrent pneumonias (secondary to obstruction), localized wheezing • Superior vena cava syndrome: 1. Obstruction of venous return of the superior vena cava is most commonly caused by bronchogenic carcinoma or metastasis to paratracheal nodes. 2. The patient usually complains of headache, nausea, dizziness, visual changes, syncope, and respiratory distress. 3. Physical examination reveals distention of thoracic and neck veins, edema of face and upper extremities, facial plethora, and cyanosis. • Horner’s syndrome: constricted pupil, ptosis, facial anhidrosis caused by spinal cord damage between C8 and T1 secondary to a superior sulcus tumor (bronchogenic carcinoma of the extreme lung apex); a superior sulcus tumor associated with ipsilateral Horner’s syndrome and shoulder pain is known as “Pancoast” tumor.

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Lung Neoplasms, Primary system for non–small cell lung cancer. In this system, stage 1 (N0 [no lymph node involvement]), stage 2 (N1 [spread to ipsilateral bronchopulmonary or hilar lymph nodes]) include localized tumors for which surgical resection is the preferred treatment. Stage 3 is subdivided into 3A (potentially resectable) and 3B. The surgical management of stage 3A disease (N2 [involvement of ipsilateral mediastinal nodes]) is controversial. Only 20% of N2 disease is considered minimal disease (involvement of only one node) and technically resectable. Stage 4 indicates metastatic disease. The pathologic staging system uses a tumor/nodal involvement/metastasis system. 2. In patients with small cell lung cancer, a more practical accepted staging system is the one developed by the Veterans Administration Lung Cancer Study Group (VALG). This system contains two stages: a. Limited stage: disease confined to the regional lymph nodes and to one hemithorax (excluding pleural surfaces) b. Extensive stage: disease spread beyond the confines of limited stage disease 3. Pretreatment staging procedures for lung cancer patients, in addition to complete history and physical examination, generally include the following tests: a. Chest x-ray (PA and lateral), ECG b. Laboratory evaluation: CBC, electrolytes, platelets, calcium, phosphorus, glucose, renal and liver function studies, ABGs, and skin tests for TB c. Pulmonary function studies d. CT scan of chest and PET scan: A recent Dutch trial revealed a 51% relative reduction in futile thoracotomies for patients with suspected non–small cell lung cancer who underwent preoperative assessment with PET with the tracer 18FDG-PET in addition to conventional workup e. Mediastinoscopy or anterior mediastinotomy in patients being considered for possible curative lung resection f. Biopsy of any accessible suspect lesions g. CT scan of liver and brain; radionuclide scans of bone in all patients with small cell carcinoma of the lung and patients

with non–small cell lung neoplasms suspected of involving these organs h. Bone marrow aspiration and biopsy only in selected patients with small cell carcinoma of the lung. In the absence of an increased LDH or cytopenia, routine bone marrow examination is not recommended

TREATMENT NONPHARMACOLOGIC THERAPY • Nutritional support • Avoidance of tobacco or other substances toxic to the lungs • Supplemental O2 prn

ACUTE GENERAL Rx NON–SMALL CELL CARCINOMA: • Surgery 1. Surgical resection is indicated in patients with limited disease (not involving mediastinal nodes, ribs, pleura, or distant sites). This represents approximately 15% to 30% of diagnosed cases. 2. Preoperative evaluation includes review of cardiac status (e.g., recent MI, major arrhythmias) and evaluation of pulmonary function (to determine if the patient can tolerate any loss of lung tissue). Pneumonectomy is possible if the patient has a preoperative FEV1 2 L or if the MVV is 50% of predicted capacity. 3. Preoperative chemotherapy should be considered in patients with more advanced disease (stage 3A) who are being considered for surgery, because it increases the median survival time in patients with non–small cell lung cancer compared with the use of surgery alone. • Treatment of unresectable non–small cell carcinoma of the lung: 1. Radiotherapy can be used alone or in combination with chemotherapy; it is used primarily for treatment of CNS and skeletal metastases, superior vena cava syndrome, and obstructive atelectasis; although thoracic radiotherapy is generally considered standard therapy for stage 3 disease, it has limited effect on survival. Palliative radiotherapy should be delayed until symptoms occur since immediate therapy offers no advantage over delayed therapy and results in more adverse events from the radiotherapy. 2. Chemotherapy: various combination regimens are available. Current

drugs of choice are paclitaxel plus either carboplatin or cisplatin; cisplatin plus vinorelbine; gemcitabine plus cisplatin; carboplatin or cisplatin plus docetaxel. The overall results are disappointing, and none of the standard regimens for non–small cell lung cancer is clearly superior to the others. Gefitinib (Iressa), an inhibitor of epidermal growth factor receptor (EGFR) tyrosine kinase, is an oral preparation currently undergoing clinical trials for advanced non–small cell lung cancer. 3. The addition of chemotherapy to radiotherapy improves survival in patients with locally advanced, unresectable non–small cell lung cancer. The absolute benefit is relatively small, however, and should be balanced against the increased toxicity associated with the addition of chemotherapy. TREATMENT OF SMALL CELL LUNG CANCER: • Limited stage disease: standard treatments include thoracic radiotherapy and chemotherapy (cisplatin and etoposide) • Extensive stage disease: standard treatments include combination chemotherapy (cisplatin or carboplatin plus etoposide or combination of irinotecan and cisplatin) • Prophylactic cranial irradiation for patients in complete remission to decrease the risk of CNS metastasis

DISPOSITION • The 5-yr survival of patients with non– small cell carcinoma when the disease is resectable is approximately 30%. • Median survival time in patients with limited stage disease and small cell lung cancer is 15 mo; in patients with extensive stage disease, it is 9 mo.

SUGGESTED READINGS Kris MG et al: Efficacy of gefitinib, an inhibitor of the epidermal growth factor receptor tyrosine kinase, in symptomatic patients with non-small cell lung cancer, JAMA 290:2149, 2003. Lardinois D et al: Staging of non-small cell lung cancer with integrated positron-emission tomography and computed tomography, N Engl J Med 348:2500, 2003. Schiller JH et al: Comparison of four chemotherapy regimens for advanced nonsmall-cell lung cancer, N Engl J Med 346:92, 2002. Spira A, Ettinger DS: Multidisciplinary management of lung cancer, N Engl J Med 350:379, 2004. AUTHOR: FRED F. FERRI, M.D.

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Lymphoma, Non-Hodgkin BASIC INFORMATION Non-Hodgkin lymphoma is a heterogeneous group of malignancies of the lymphoreticular system. NHL ICD-9CM CODES 201.9 Lymphoma, non-Hodgkin

EPIDEMIOLOGY & DEMOGRAPHICS • Sixth most common neoplasm in the U.S. (56,000 new cases/yr) • Increasing incidence with age

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients often present with asymptomatic lymphadenopathy. • Approximately one third of NHL originates extranodally. Involvement of extranodal sites can result in unusual presentations (e.g., GI tract involvement can simulate PUD). • NHL cases associated with HIV occur predominantly in the brain. • Pruritus, fever, night sweats, weight loss are less common than in Hodgkin’s disease. • Hepatomegaly and splenomegaly may be present.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Hodgkin’s disease Viral infections Metastatic carcinoma A clinical algorithm for evaluation of lymphadenopathy is described in Section III • The differential diagnosis of lymphadenopathy is described in Section II

WORKUP Initial laboratory evaluation may reveal only mild anemia and elevated LDH and ESR. Proper staging of non-Hodgkin’s lymphoma requires the following: • A thorough history, physical examination, and adequate biopsy • Routine laboratory evaluation (CBC, ESR, urinalysis, LDH, BUN, creatinine, serum calcium, uric acid, LFTs, serum protein electrophoresis) • Chest x-ray examination (PA and lateral) • Bone marrow evaluation (aspirate and full bone core biopsy) • CT scan of abdomen and pelvis; CT scan of chest if chest x-ray films abnormal • Bone scan (particularly in patients with histiocytic lymphoma)

IMAGING STUDIES See “Workup.”

TREATMENT ACUTE GENERAL Rx The therapeutic regimen varies with the histologic type and pathologic stage. Following are the commonly used therapeutic modalities: LOW-GRADE NHL (E.G., NODULAR, POORLY DIFFERENTIATED): 1. Local radiotherapy for symptomatic obstructive adenopathy 2. Deferment of therapy and careful observation in asymptomatic patients 3. Single-agent chemotherapy with cyclophosphamide or chlorambucil and glucocorticoids 4. Combination chemotherapy alone or with radiotherapy: generally indicated only when the lymphoma becomes more invasive, with poor response to less aggressive treatment; commonly used regimens: CVP, CHOP, CHOPBLEO, COPP, BACOP; addition of recombinant alpha interferon at low doses to chemotherapy prolongs remission duration in patients with lowgrade NHL 5. Monoclonal antibodies directed against B-cell surface antigens can also be used to treat follicular lymphomas that are resistant to conventional therapy. The anti-CD20 monoclonal antibody rituximab is a targeted, minimally toxic treatment effective against low-grade NHL in patients who have not received previous treatment 6. The addition of rituximab to CHOP is generally well tolerated; however, ad-

DISEASES AND DISORDERS

SYNONYMS

ditional studies may be necessary to clarify the role of CHOP plus rituximab in patients with indolent NHL 7. Ibritumomab tiuxetan (Zevalin), an immunoconjugate that combines the linker-chelator tiuxetan with the monoclonal antibody ibritumomab, can be used as part of a two-step regimen for treatment of patients with relapsed or refractory low-grade, follicular, or transformed B-cell NHL refractory to rituximab 8. New purine analogs (FLAMP, 2CDA) can be used in salvage treatment of refractory lymphomas. They all have activity in follicular lymphomas INTERMEDIATE- AND HIGH-GRADE LYMPHOMAS (E.G., DIFFUSE HISTIOCYTIC LYMPHOMA): Combination chemotherapy regimens (e.g., CHOP, PRO-MACE-CYTABOM, MACOP-B, M-BACOD). An anthracycline-containing regimen (such as CHOP) given in standard doses and schedule is generally best for treatment of older patients with advanced stage, aggressive-histology lymphoma who do not have significant comorbid illness. 1. High-dose sequential therapy is superior to standard-dose MACOP-B for patients with diffuse large-cell lymphoma of the B-cell type. 2. Dose-modified chemotherapy should be considered for most HIV-infected patients with lymphoma. As compared with treatment with standard doses of cytotoxic chemotherapy (MBACOD), reduced doses cause significantly fewer hematologic toxic effects yet have similar efficacy in patients with HIV-related lymphoma. • Three cycles of CHOP followed by involved-field radiotherapy may be superior to eight cycles of CHOP alone in patients with localized intermediate- and high-grade NHL. • The addition of rituximab against CD20 B-cell lymphoma to the CHOP regimen increases the complete response rate and prolongs event-free and overall survival in elderly patients with diffuse large B-cell lymphoma without a clinically significant increase in toxicity. Bexxar, a combination of the mononuclear antibody tositumomab and radiolabeled iodine-131 tositumomab can be used for a single treatment of relapsed follicular NHL in patients who are refractory to rituximab. It results in complete remission in 25% of patients and clinical response in 60% of patients. • Granulocyte colony-stimulating factor (G-CSF): may be effective in reducing the risk of infection in patients with aggressive lymphoma undergoing chemotherapy

Section I

DEFINITION

• Depending on the histopathology, the results of the above studies and the planned therapy, some other tests may be performed: gallium scan (e.g., in patients with high-grade lymphomas), liver/spleen scan, PET scan, lymphangiography, lumbar puncture • -2 Microglobulin levels should be obtained initially (prognostic value) and serially in patients with low-grade lymphomas (useful to monitor therapeutic response of the tumor) • Serum interleukin levels have prognostic value in diffuse large cell lymphoma CLASSIFICATION: The Working Formulation of non-Hodgkin lymphoma for clinical usage subdivides lymphomas into low grade, intermediate grade, high grade, and miscellaneous (Table 1-9). STAGING: The Ann Arbor classification is used to stage non-Hodgkin lymphomas, and histopathology has greater therapeutic implications in NHL than in Hodgkin’s disease.

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Lymphoma, Non-Hodgkin • Radioimmunotherapy with (131I) anti-B1 antibody therapy for NHL either by itself or in combination with other treatments • Treatment with high-dose chemotherapy and autologous bone marrow transplant: as compared with conventional chemotherapy, increases event-free and overall sur-

TABLE 1-9

vival in patients with chemotherapysensitive non-Hodgkin lymphoma in relapse

DISPOSITION • Patients with low-grade lymphoma, despite their long-term survival (6 to 10 yr average), are rarely cured, and the great majority (if not all) eventually die of the

lymphoma, whereas patients with a high-grade lymphoma may achieve a cure with aggressive chemotherapy. • Complete remission occurs in 35% to 50% of patients with intermediate- and high-grade lymphoma. Prognostic factors include the histiologic subtype, age of patient, and bulk of disease. AUTHOR: FRED F. FERRI, M.D.

Classification Systems for Grading Lymphomas

Kiel Classification

Working Formulation

Low-grade malignancy Lymphocytic, CLL Lymphocytic, other Lymphoplasmacytoid Centrocytic

Low grade A. Malignant lymphoma, small lymphocytic Consistent with chronic lymphocytic leukemia

Centroblastic/Centrocytic Follicular without sclerosis Follicular with sclerosis Follicular and diffuse, without sclerosis Follicular and diffuse, with sclerosis Diffuse Low-grade malignant lymphoma, unclassified High-grade malignancy Centroblastic Lymphoblastic, Burkitt’s type Lymphoblastic, convoluted cell type Lymphoblastic, other (unclassified) immunoblastic High-grade malignant lymphoma, unclassified Malignant lymphoma unclassified (unable to specify high grade or low grade) Composite lymphoma

Revised European-American Classification B-cell lymphomas B-CLL/SLL Lymphoplasmacytoid lymphoma Follicle center lymphomas

B. Malignant lymphoma, follicular, predominantly small cleaved cell

Marginal zone lymphomas (MALT) Mantle cell lymphoma

Diffuse areas Sclerosis C. Malignant lymphoma, follicular mixed, small cleaved and large cell Diffuse areas

Diffuse large B-cell lymphoma

Sclerosis

Primary mediastinal large B-cell lymphoma Burkitt’s lymphoma

Intermediate grade D. Malignant lymphoma, follicular Diffuse areas E. Malignant lymphoma, diffuse small cleaved cell

T-cell lymphomas T-CLL Mycosis fungoides/Sézary syndrome

F. Malignant lymphoma, diffuse mixed, small and large cell sclerosis G. Malignant lymphoma diffuse Large cell Cleaved cell Noncleaved cell Sclerosis High grade H. Malignant lymphoma large cell, immunoblastic Plasmacytoid Clear cell Polymorphous Epithelioid cell component I. Malignant lymphoma lymphoblastic Convoluted cell Nonconvoluted cell J. Malignant lymphoma small noncleaved cell Burkitt’s Follicular areas

Peripheral T-cell lymphoma, unspecified Angioimmunoblastic T-cell lymphoma Angiocentric lymphoma Intestinal T-cell lymphoma Adult T-cell lymphoma/leukemia Anaplastic large cell lymphoma Precursor T-lymphoid lymphoma/leukemia

From Abeloff MD: Clinical oncology, ed 2, New York, 2000, Churchill Livingstone. B-CLL, B-cell chronic lymphoid leukemia; MALT, mucosa-associated lymphoid tumor; SLL, lymphoid leukemia; T-CLL, T-cell CLL.

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Macular Degeneration BASIC INFORMATION

ICD-9CM CODES 362.5 Degeneration of macula and posterior pole

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • Main cause of blindness in the U.S. in 40 yr and older • Increases with age • 1.75 million individuals in the U.S. currently affected; 3 million by year 2020 PREDOMINANT SEX: Male  female (15% of white women 80 have severe ARMD) PREDOMINANT AGE: 50 yr PEAK INCIDENCE: • 75 to 80 yr old • Dramatic increases in incidence and prevalence with age until approximately 80% of people 75 yr or older have senile macular degeneration. GENETICS: • Different syndrome: senile macular degeneration is age related. • Several rare neurologic syndromes are associated with macular degeneration. • Vascular disease closely related to macular degeneration.

PHYSICAL FINDINGS • Decreased central vision • Macular hemorrhage, pigmentation, edema, atrophy • The most common abnormality seen in age-related macular degeneration (AMD) is the presence of drusen, or yellowish deposits deep to the retina; this may be early in course of disease

• Follow closely by ophthalmologist. • If vision deteriorates, refer urgently to an ophthalmologist.

DIAGNOSIS

PEARLS & CONSIDERATIONS

DIFFERENTIAL DIAGNOSIS • • • •

Diabetic retinopathy Hypertension Histoplasmosis Trauma with scar

WORKUP • Complete eye examination, including visual field and fluorescein angiography • Optical coherence tomography (OCT)

LABORATORY TESTS Evaluate for diabetes and other metabolic problems, as well as vascular diseases.

IMAGING STUDIES • Optical coherence tomography (OCT) • Fluorescein angiography

TREATMENT NONPHARMACOLOGIC THERAPY • Laser treatment to stop progression of disease—photo dynamic treatment with verteporfin IV • Laser (Argon) • Diet, exercise • Vitamins with zinc and antioxidants

ACUTE GENERAL Rx Intravitral steroids, photodynamic treatment (PDT) with laser

CHRONIC Rx • Repeated laser treatments • Antioxidants and zinc may slow down progression of ARMD

REFERRAL • To ophthalmologist early in the course of the disease if the sight is to be saved • Immediate referral if any change in vision

COMMENTS • Sildenafil has no significant effect on macular degeneration. • The vision of only 1 out of 10 people can be saved, but the disease is so devastating that vigorous therapy should be attempted. • Statins plus aspirin may slow down progression. • Vitamins with zinc and antioxidants may slow down progression of ARMD.

SUGGESTED READINGS Friedman DS et al: Prevalence of age-related macular degeneration in the US, Arch Ophthalmol 122(4):564, 2004. Gottlieb JL: Age-related macular degeneration, JAMA 288:2233, 2002. Jonas JB: Verteporfin theory of subfoveal choroidal neovascularization in age-related macular degeneration, Am J Ophthalmol 133(6):F57, 2002. Liu M, Regillo CD: A review of treatments for macular degeneration: a synopsis of currently approved treatments and ongoing clinical trials, Curr Opin Ophthalmol 15(3):221, 2004. Makenzie PJ, Chang TS: ETN assessment of vision-related emotion in patients with age related macular degeneration, Ophthalmology 109(4):720, 2002. Ting TD et al: Decreased visual acuity associated with cystoid macular edema in neovascular or age-related macular degeneration, Arch Ophthamol 120(6):731, 2002. AUTHOR: MELVYN KOBY, M.D.

DISEASES AND DISORDERS

Macular degeneration refers to a group of diseases associated with loss of central vision and damage to the macula. Degenerative changes occur in the pigment, neural, and vascular layers of the macula. The dry macular degeneration is usually ischemic in etiology, and a wet macular degeneration is associated with leakage of fluid from blood vessels, usually referred to as age-related macular degeneration (ARMD).

DISPOSITION

• Subretinal neovascular membrane early • Pigmentary and vascular changes with exudate, edema, and scar tissue development • Early in course, possible subretinal neovascularization • Dry type atrophy of macular pigment epithelium

Section I

DEFINITION

ETIOLOGY

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Meniere’s Disease BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Meniere’s disease is a syndrome characterized by recurrent vertigo with fluctuating hearing loss, tinnitus, and fullness in the ear.

• • • • • • •

SYNONYMS Endolymphatic hydrops Meniere’s syndrome ICD-9CM CODES 386.01 Meniere’s disease, cochleovestibular (active)

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 15 cases/100,000 persons PREVALENCE (IN U.S.): 100-200 cases/ 100,000 persons PREDOMINANT SEX: Male  female PREDOMINANT AGE: Adults GENETICS: Not known to be genetic

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Hearing may be unilaterally decreased • Pallor, sweating, and nausea may occur during a severe attack • Usually the patient develops a sensation of fullness and pressure along with decreased hearing and tinnitus in a single ear • The patient typically experiences severe vertigo, which peaks within minutes, then slowly subsides over hours • Persistent sense of disequilibrium for days is typical after an acute episode

ETIOLOGY • Unknown; viral and autoimmune etiologies have been suggested • Associated with endolymphatic hydrops

Acoustic neuroma Migrainous vertigo Multiple sclerosis Autoimmune inner ear syndrome Otitis media Vertebrobasilar disease Viral labyrinthitis

WORKUP • Electronystagmography may show peripheral vestibular deficit. • Electrocochleography and glycerol test used by some otoneurologists and ENT specialists.

LABORATORY TESTS Audiogram may show sensorineural hearing loss, with lower frequencies primarily affected.

IMAGING STUDIES MRI to rule out acoustic neuroma, especially if cerebellar or CNS dysfunction is present

TREATMENT NONPHARMACOLOGIC THERAPY Limit activity during attacks.

ACUTE GENERAL Rx • Prochlorperazine 5 to 10 mg PO q6h or 25 mg PO bid • Promethazine 12.5 to 25 mg PO q4-6h • Diazepam 5 to 10 mg IV/PO for acute attack • Meclizine 12.5 to 25 mg tid • Scopolamine patch

CHRONIC Rx Diuretics such as hydrochlorothiazide or acetazolamide, salt restriction, and avoidance of caffeine are traditional.

DISPOSITION • Usual course of disease consists of alternating attacks and remissions • Majority of patients can be managed medically; fewer than 10% of patients will undergo surgical intervention for persistent incapacitating vertigo

REFERRAL To an otolaryngologist for surgical intervention if attacks persist despite medical therapy

PEARLS & CONSIDERATIONS COMMENTS • Many variations of the classical clinical picture. The essential features for diagnosis are episodic vertigo and sensorineural hearing loss audiometrically documented on at least one occasion. • In one-third of patients both ears are eventually involved. • Some evidence that Meniere’s disease and migraine may be pathophysiologically linked.

SUGGESTED READINGS Baloh RW, Fife TD, Furman JM, Zee DS: Recurrent spontaneous attacks of vertigo, Continuum Lifelong Learning in Neurology 2(2):56, 1996. Radtke A et al: Migraine and Meniere’s disease: is there a link? Neurology 59(11):1700, 2002. Thai-Von H, Bounaix MJ, Fraysse B: Meniere’s disease: pathophysiology and treatment, Drugs 61(8):1089, 2001. Weber PC, Adkins WY Jr: The differential diagnosis of Meniere’s disease, Otolaryngol Clin North Am 30(6):977, 1997. AUTHOR: SHARON S. HARTMAN, M.D., PH.D.

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Menopause BASIC INFORMATION

SYNONYMS Change of life Climacteric ovarian failure ICD-9CM CODES 627.2 Menopausal or female climacteric states 627.4 States associated with artificial menopause 716.3 Climacteric arthritis

EPIDEMIOLOGY & DEMOGRAPHICS • Average age of menopause in the U.S. is 51 yr. • Age at which menopause occurs is genetically determined. • Smokers experience menopause an average of 1.5 yr earlier than nonsmokers. • More than one third of a woman’s life will be spent after menopause. • Onset of perimenopause is usually in a woman’s mid- to late 40s. • Approximately 4000 women each day begin menopause.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Atrophic vaginitis, which can cause burning, itching, bleeding, dyspareunia • Either complete cessation of menses or a period of irregular cycles and diminished or heavier bleeding • Osteoporosis • Psychologic dysfunction: 1. Anxiety 2. Depression 3. Insomnia 4. Nervousness 5. Irritability 6. Inability to concentrate • Sexual changes, decreased libido, dyspareunia • Urinary incontinence • Vasomotor symptoms (hot flashes, flushes), night sweats, cardiovascular disease, coronary artery disease, atherosclerosis, headaches, tiredness, and lethargy

• TSH to rule out thyroid dysfunction and prolactin level if patient has symptoms of galactorrhea and if suspicion of pituitary adenoma exists • A general chemistry profile to check for any systemic diseases • Pap smear, endometrial biopsy, or D&C in patients who have had irregular periods or intermenstrual or postmenopausal bleeding • Mammogram

IMAGING STUDIES • CT scan or MRI of head if pituitary tumor is suspected • Bone density studies • Pelvic ultrasound to check endometrial stripe

DIAGNOSIS

TREATMENT

DIFFERENTIAL DIAGNOSIS

NONPHARMACOLOGIC THERAPY

• • • • • • • • •

Asherman’s syndrome Hypothalamic dysfunction Hypothyroidism Pituitary tumors Adrenal abnormalities Ovarian abnormalities Polycystic ovarian syndrome Ovarian neoplasm TB

WORKUP • If the clinical picture is highly suggestive of menopause, estrogen can be prescribed. If all symptoms resolve, then diagnosis has essentially been made. Before estrogen is prescribed, a complete history and physical examination are needed. If a patient has estrogendependent malignancy, unexplained abnormal uterine bleeding, history of thrombophlebitis, or acute liver disease, estrogen therapy is contraindicated. • Progesterone challenge test: progesterone 100 mg is given IM to induce withdrawal bleeding. If no withdrawal bleeding is obtained, it would be safe to assume that a hypoestrogenic state is present. • Physical examination, height, weight, blood pressure, breast examination, and pelvic examination are needed. • Assess risk for coronary artery disease, osteoporosis, cigarette smoking, personal history, history of breast cancer, liver disease, active coagulation disorder, or any unexplained vaginal bleeding.

LABORATORY TESTS • FSH, LH, and estrogen levels: if the FSH is markedly elevated and the estrogen level is markedly depressed, constitutes laboratory diagnosis of ovarian failure

• A balanced diet: low in fat, with total fat intake being 30% of calories; total calories sufficient to maintain body weight or to produce weight loss if that is needed • Avoidance of smoking, excessive alcohol or caffeine intake • Exercise: weight-bearing exercise for osteoporosis prevention • Kegel exercises for strengthening the pelvic floor • Adequate calcium intake: 1500 mg qd and vitamin D 600 units qd are necessary to maintain calcium balance in postmenopausal women and prevent osteoporosis • Change in the ambient temperature (may ameliorate hot flashes and reduce night sweats) • Vitamin E • Avoidance of caffeine, alcohol, and spicy foods if they trigger hot flashes • Vaginal lubricants to help with the dyspareunia secondary to vaginal dryness (e.g., Replens, K-Y Jelly, or GyneMoistrin cream)

ACUTE GENERAL Rx Estrogen replacement in symptomatic patients can be done in a variety of forms, including oral estrogen and transdermal estrogen patch. • Examples of oral estrogen would include conjugated estrogens: 1. Premarin: start with 0.625 mg qd and increase up to 1.25 mg qd, depending on symptoms. Cenestin (synthetic conjugated estrogens, A) available in 0.625-, 0.9-, and 1.25mg doses. 2. Estradiol (Estrace): start with 1 mg qd and increase to 2 mg qd; also

DISEASES AND DISORDERS

Menopause is the occurrence of no menstrual periods for 1 yr after age 40 yr or permanent cessation of ovulation following lost ovarian activity. It is a climacteric reproductive stage of life marked by waxing and waning estrogen levels followed by decreasing ovarian function. Premature ovarian failure and no menstrual periods may also occur because of depletion of ovarian follicles before the age of 40 yr.

• The most common etiology: physiologic, caused by degenerating theca cells that fail to react to endogenous gonadotropins, producing less estrogen; decreased negative feedback in the hypothalamic pituitary access, increased follicle-stimulating hormone (FSH), and increased luteinizing hormone (LH), which leads to stromal cells that continue to produce androgens as a result of the LH stimulation • Surgical castration • Family history of early menopause, cigarette smoking, blindness, abnormal chromosomal karyotype (Turner’s syndrome, gonadal dysgenesis), precocious puberty, and left-handedness

Section I

DEFINITION

ETIOLOGY

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Menopause

•

•

•

•

available in 0.5 mg tablet for patients who experience side effects from the estrogen. 3. Esterified estrogens (Estratab): start with 0.3 to 1.25 mg qd. 4. Estropipate (Ogen, Ortho-Est): start with 0.625 to 1.25 mg qd. 5. Esterified estrogen/testosterone combination: give 1.25 mg and methyltestosterone 2.5 mg (Estratest) and esterified estrogen 0.625 mg and methyltestosterone 1.25 mg (Estratest HS). May improve sexual enjoyment and libido. If the patient has had a hysterectomy for benign disease, estrogen alone is sufficient. However, if she still has her uterus, progestin should be added for its protective effect against endometrial cancer. Medroxyprogesterone acetate (Provera) is the most commonly prescribed progestin. It can be prescribed in a continual daily dose of 2.5 mg or of 5 mg if continual breakthrough bleeding is encountered. This can also be prescribed in a 5-mg cyclic fashion for the first 14 days of the month or as 10-mg tablets for the first 10 days of the month. Patients need to be advised that this generally will cause withdrawal bleeding but in a fairly regular fashion. Continuous hormone replacement therapy is preferred, because after a period of time the patient should be amenorrheic. Patients should be counseled that they may experience some irregular spotting for the first 6 to 9 mo after starting the hormone replacement therapy. Combination oral preparations Femhrt 1 ⁄5 (1 mg norethindrone acetate/5 g ethinyl estradiol) one pill daily. Ortho— Prefest 1 mg 17-estradiol (white pill) alternating with 1 mg 17-estradiol and 0.9 mg norgestimate (pink pill) q3d Prempro 0.625 mg conjugated estrogen/2.5 mg medroxyprogesterone one pill daily Prempro 0.625 mg conjugated estrogen/5.0 mg medroxyprogesterone one pill daily Activella 1 mg estradiol and 0.5 mg norethindrone acetate Premphase 0.625 mg conjugated estrogen with 5 mg medroxyprogesterone last 14 days. Transdermal patches can be either estradiol (Estraderm, Vivelle, Fempatch) 0.025 to 0.1 mg applied twice weekly or Climara 0.025 to 0.1 mg used once a week. With these preparations, progesterone should be used in a similar fashion. Combipatch— apply twice weekly (combination estrogen and progesterone). Vaginal creams can be used, and these should be reserved for local therapy of atrophic vaginitis. Systemic absorption does occur; however, blood levels are unpredictable. Start with a loading dose of 2 to 4 g of estrogen-containing

•

• • •

•

cream nightly for 1 to 2 wk. When symptoms improve, once to twice weekly is adequate maintenance. Vagifem estradiol vaginal tablets. Initial dosage: one Vagifem tablet, inserted vaginally, once daily for 2 wk. Maintenance dose: one Vagifem tablet, inserted vaginally, twice weekly. Femring vaginal ring delivering the equivalent of 50 micrograms per day inserted every 3 months. EstroGel 0.06% (estrodiol gel) One Pump (1.25 g/day) applied to one arm from wrist to shoulder. For women in whom estrogen is contraindicated or for those who do not wish to take estrogen, the following regimens can be used: 1. Depo-Provera 150 mg IM every month (may be helpful in alleviating hot flashes) 2. Clonidine 0.05 to 0.15 mg qd 3. Bellergal-S 4. Fosamax (alendronate sodium) or Actonel (risedronate) 5 mg qd or 35 mg weekly are approved for prophylactic prevention of osteoporosis. They should be taken on an empty stomach; wait at least 30 min before ingesting any substance, including liquids, because this decreases absorption into the body. They should be swallowed on arising for the day with a full glass of water, 6 to 8 oz, and patients should not lie down for at least 30 min and until after their first food of the day. 5. Evista (Raloxifene) 60 mg daily PO has positive bone effect, a total cholesterol–lowering effect, and LDL cholesterol–lowering effect; it is a selective estrogen receptor agonist; it does not affect estrogen receptors in the breast or uterus. It does not ameliorate vasomotor symptoms or vaginal atrophy. Tibolone significantly improves vasomotor symptoms, libido, and vaginal lubrication.

CHRONIC Rx Hormone replacement therapy should be used only for the short term unless benefits outweigh the risks of long-term use.

DISPOSITION If treated, the patient should have resolution of her symptoms and reduced incidence of osteoporosis. Lifelong medical supervision is necessary to monitor adequacy of treatment and prevention of complications. This should include annual Pap smears, pelvic examinations, breast examinations, mammography, and endometrial sampling of any type of abnormal bleeding. If untreated, the vasomotor symptoms will eventually disappear; however, this takes many years, and some

women who are in their 80s have experienced hot flashes. Urogenital atrophy will continue to worsen. Osteoporosis and coronary artery disease risks will increase with every passing year.

REFERRAL This condition can be managed adequately by the patient’s primary care physician who has an interest in treating menopausal women.

PEARLS & CONSIDERATIONS COMMENTS • Short-term risks of HRT include an 18fold increased risk for cholecystitis, 3.5-fold risk of a thrombocardiac event in the first year, and probably increased risk of stroke and MI. • Results of the WHI study found that for every 10,000 women taking HRT for 1 yr (10,000 person-yr), 7 more would have coronary events, 8 more strokes, 8 more pulmonary emboli, and 8 more with early breast cancer than would 10,000 women taking placebo. Benefits of HRT were 6 fewer cases of colorectal cancer and 5 fewer hip fractures per 10,000 women. • HRT should not be initiated or continued for the primary or secondary prevention of CHD or osteoporosis. • Patient education materials can be obtained through the American College of Obstetricians and Gynecologists, 409 12th Street SW, Washington, DC 20024, and Menopause News, 2074 Union Street, San Francisco, CA 94123; phone: 1-800-241-MENO. Multiple patient educational brochures are produced by pharmacologic companies.

SUGGESTED READINGS Gambrell RD: The Women’s Health Initiative Reports: critical review of the findings, the female patient, Menopause 29:(11):23, 2004. Han KK et al: Benefits of soy isoflavone therapeutic regimen on menopausal symptoms, Obstet Gynecol 99:389, 2002. Lacey JV et al: Menopausal hormone replacement therapy and risk of ovarian cancer, JAMA 288:334, 2002. Nelson H et al: Postmenopausal hormone replacement therapy, JAMA 288:872, 2002. Santoro N: The menopause transition: an update, Human Reproduction Update 8(2):155, 2002. Speroff L: Efficacy and tolerability of a novel estradiol vaginal ring for relief of menopausal symptoms, Obstet Gynecol 102(4):823, 2003. Writing Group for the Women’s Health Initiative Investigators: Risks and benefits of estrogen plus progestin in healthy postmenopausal women, JAMA 288:321, 2002. AUTHOR: GEORGE T. DANAKAS, M.D.

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Mitral Regurgitation

DEFINITION

DIFFERENTIAL DIAGNOSIS

Mitral regurgitation (MR) is retrograde blood flow through the left atrium secondary to an incompetent mitral valve. Eventually there is an increase in left atrial and pulmonary pressures, which may result in right ventricular failure.

• • • •

Hypertrophic cardiomyopathy Pulmonary regurgitation Tricuspid regurgitation VSD

WORKUP

SYNONYMS

Diagnostic workup consists of echocardiography, ECG, and chest x-ray.

Mitral insufficiency MR

IMAGING STUDIES

ICD-9CM CODES 424.0 Mitral regurgitation

EPIDEMIOLOGY & DEMOGRAPHICS The incidence of MR has increased over the past 30 yr; however, this may be because of increasing availability of echocardiography rather than any real increases in this condition.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients with MR generally present with the following symptoms: 1. Fatigue, dyspnea, orthopnea, frank CHF 2. Hemoptysis (caused by pulmonary hypertension) 3. Possible systemic emboli in patients with left atrial mural thrombi associated with atrial fibrillation • Hyperdynamic apex, often with palpable left ventricular lift and apical thrill • Holosystolic murmur at apex with radiation to base or to left axilla; poor correlation between the intensity of the systolic murmur and the degree of regurgitation • Apical early- to mid-diastolic rumble (rare)

ETIOLOGY • Papillary muscle dysfunction (as a result of ischemic heart disease) • Ruptured chordae tendineae • Infective endocarditis • Calcified mitral valve annulus • Left ventricular dilation • Rheumatic valvulitis • Primary or secondary mitral valve prolapse • Hypertrophic cardiomyopathy • Idiopathic myxomatous degeneration of the mitral valve • Myxoma • SLE

• Echocardiography: enlarged left atrium, hyperdynamic left ventricle (erratic motion of the leaflet is seen in patients with ruptured chordae tendineae); Doppler electrocardiography will show evidence of MR. The most important aspect of the echocardiographic examination is the quantification of left ventricular systolic performance. • Chest x-ray study: 1. Left atrial enlargement (usually more pronounced in mitral stenosis) 2. Left ventricular enlargement 3. Possible pulmonary congestion • ECG: 1. Left atrial enlargement 2. Left ventricular hypertrophy 3. Atrial fibrillation

TREATMENT NONPHARMACOLOGIC THERAPY Salt restriction

ACUTE GENERAL Rx • Medical: Medical therapy is primarily directed toward treatment of complications (e.g., atrial fibrillation) and prevention of bacterial endocarditis. 1. Consider digitalis for inotropic effect and to control ventricular response if atrial fibrillation with fast ventricular response is present 2. Afterload reduction (to decrease the regurgitant fraction and to increase cardiac output): may be accomplished with nifedipine, hydralazine plus nitrates, ACE inhibitors, or RRBs 3. Anticoagulants if atrial fibrillation occurs 4. Antibiotic prophylaxis before dental and surgical procedures

• Surgery: Surgery is the only definitive treatment for MR. Transesophageal echocardiography allows accurate assessment of the feasibility of valve repair and is indicated before surgical intervention. The timing of surgical repair is controversial; generally surgery should be considered early in symptomatic patients despite optimal medical therapy and in patients with moderate to severe MR and minimal symptoms if there is echocardiographic evidence of rapidly progressive increase in left ventricular enddiastolic and end-systolic dimension (echocardiographic evidence of systolic failure includes end-systolic dimension 55 mm and fractional shortening 31%). Surgery is also indicated in asymptomatic patients with preserved ventricular function if there is a high likelihood of valve repair or if there is evidence of pulmonary hypertension or recent atrial fibrillation.

DISPOSITION Prognosis is generally good unless there is significant impairment of left ventricle or significantly elevated pulmonary artery pressures. Most patients remain asymptomatic for many years (average interval from diagnosis to onset of symptoms is 16 yr).

REFERRAL Surgical referral in selected patients (see “Acute General Rx”); emergency surgery may be necessary in patients with MR caused by ruptured chordae tendineae following MI.

PEARLS & CONSIDERATIONS COMMENTS Patients should be counseled regarding weight reduction (if obese), avoidance of tobacco, and maintenance of normal (nonstrenuous) activities.

SUGGESTED READING Otto CM: Evaluation and management of chronic mitral regurgitation, N Engl J Med 345:740, 2001. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

DIAGNOSIS

Section I

BASIC INFORMATION

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Mitral Stenosis BASIC INFORMATION DEFINITION Mitral stenosis is a narrowing of the mitral valve orifice. The cross section of a normal orifice measures 4 to 6 cm2. A murmur becomes audible when the valve orifice becomes smaller than 2 cm2. When the orifice approaches 1 cm2, the condition becomes critical, and symptoms become more evident.

SYNONYMS MS ICD-9CM CODES 394.0 Mitral stenosis

• Right ventricular dysfunction (in late stages) may be manifested by peripheral edema, enlarged and pulsatile liver, and ascites.

ETIOLOGY • Progressive fibrosis, scarring, and calcification of the valve • Rheumatic fever (still a common cause in underdeveloped countries); heart valves most frequently affected in rheumatic heart disease (in descending order of occurrence): mitral, aortic, tricuspid, and pulmonary • Rare causes: endomyocardial fibroelastosis, malignant carcinoid syndrome, SLE

EPIDEMIOLOGY & DEMOGRAPHICS

DIAGNOSIS

• The occurrence of mitral valve stenosis has decreased worldwide over the past 30 yr (particularly in developed countries) as a result of declining incidence of rheumatic fever. • The incidence of mitral stenosis is higher in women.

DIFFERENTIAL DIAGNOSIS

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Exertional dyspnea initially, followed by orthopnea and PND • Acute pulmonary edema (may develop after exertion) • Systemic emboli (caused by stagnation of blood in the left atrium; may occur in patients with associated atrial fibrillation) • Hemoptysis (may be present as a result of persistent pulmonary hypertension) • Prominent jugular A waves are present in patients with normal sinus rhythm. • Opening snap occurs in early diastole; a short (0.07-second) A2 to opening snap interval indicates severe mitral stenosis. • Apical middiastolic or presystolic rumble that does not radiate is present. • Accentuated S1 (because of delayed and forceful closure of the valve) is present. • If pulmonary hypertension is present, there may be an accentuated P2 and/or a soft, early diastolic decrescendo murmur (Graham Steell murmur) caused by pulmonary regurgitation (it is best heard along the left sternal border and may be confused with aortic regurgitation). • A palpable right ventricular heave may be present at the left sternal border. • Patients with mitral stenosis usually have symptoms of left-sided heart failure: dyspnea on exertion, PND, orthopnea.

• Left atrial myxoma • Other valvular abnormalities (e.g., tricuspid stenosis, mitral regurgitation) • Atrial septal defect

WORKUP Physical examination and echocardiography

IMAGING STUDIES • Echocardiography: 1. The characteristic finding on echocardiogram is a markedly diminished E to F slope of the anterior mitral valve leaflet during diastole; there is also fusion of the commissures, resulting in anterior movement of the posterior mitral valve leaflet during diastole (calcification in the valve may also be noted). Two-dimensional echocardiogram can accurately establish valve area. • Chest x-ray: 1. Straightening of the left cardiac border caused by dilated left atrial appendage 2. Left atrial enlargement on lateral chest x-ray (appearing as double density of PA chest x-ray) 3. Prominence of pulmonary arteries 4. Possible pulmonary congestion and edema (Kerley B lines) • ECG: 1. Right ventricular hypertrophy; right axis deviation caused by pulmonary hypertension 2. Left atrial enlargement (broad notched P waves) 3. Atrial fibrillation • Cardiac catheterization to help establish the severity of mitral stenosis and

diagnose associated valvular and coronary lesions. Findings on cardiac catheterization include: 1. Normal left ventricular function 2. Elevated left atrial and pulmonary pressures

TREATMENT NONPHARMACOLOGIC THERAPY Decrease level of activity in symptomatic patients.

ACUTE GENERAL Rx • Medical: 1. If the patient is in atrial fibrillation, control the rate response with diltiazem, digitalis, or esmolol. Although digitalis is the drug of choice for chronic heart rate control, IV diltiazem or esmolol may be acutely preferable when a rapid decrease in heart rate is required. 2. If the patient has persistent atrial fibrillation (because of large left atrium), permanent anticoagulation is indicated to decrease the risk of serious thromboembolism. 3. Treat CHF with diuretics and sodium restriction. 4. Give antibiotic prophylaxis with dental and surgical procedures (see Section V). • Surgical: valve replacement is indicated when the valve orifice is 0.7 to 0.8 cm2 or if symptoms persist despite optimal medical therapy; commissurotomy may be possible if the mitral valve is noncalcified and if there is pure mitral stenosis without significant subvalvular disease. • Percutaneous transvenous mitral valvotomy (PTMV) is becoming the therapy of choice for many patients with mitral stenosis responding poorly to medical therapy, particularly those who are poor surgical candidates and whose valve is not heavily calcified; balloon valvotomy gives excellent mechanical relief, usually resulting in prolonged benefit.

DISPOSITION • Prognosis is generally good except in patients with chronic pulmonary hypertension. • Operative mortality rates for mitral valve replacement are 1% to 5% in most institutions. AUTHOR: FRED F. FERRI, M.D.

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Mitral Valve Prolapse BASIC INFORMATION

SYNONYMS

DIFFERENTIAL DIAGNOSIS

MVP Mitral click murmur syndrome

• Other valvular abnormalities • Constrictive pericarditis • Ventricular aneurysm

Monitoring for complications: • Bacterial endocarditis (risk is three to eight times that of the general population) • TIA or stroke secondary to embolic phenomena (from fibrin and platelet thrombi) • Cardiac arrhythmias (usually supraventricular) • Sudden death (rare occurrence, most often caused by ventricular arrhythmias) • Mitral regurgitation (most common complication of MVP)

WORKUP

DISPOSITION

• Medical history and physical examination • Workup consists primarily of echocardiography in patients with a systolic click or murmur on careful auscultation

The incidence of complications of MVP is very low (1%/yr) and generally associated with an increase in mitral leaflet thickness to 5 mm.

REFERRAL

IMAGING STUDIES

Surgical referral may be necessary in patients who develop symptomatic progressive mitral regurgitation.

DEFINITION Mitral valve prolapse (MVP) is the posterior bulging of interior and posterior leaflets in systole. Mitral valve prolapse syndrome refers to a constellation of MVP and associated symptoms (e.g., autonomic dysfunction, palpitations) or other physical abnormalities (e.g., pectus excavatum).

ICD-9CM CODES 424.0 Mitral valve disorders 394.9 Other and unspecified mitral valve diseases

EPIDEMIOLOGY & DEMOGRAPHICS • MVP can be found by 2-D echocardiogram in 4% of the general population (females  males). • Increased incidence is seen with autoimmune thyroid disorders, EhlersDanlos syndrome, Marfan’s syndrome, pseudoxanthoma elasticum, pectus excavatum, anorexia nervosa, and bulimia.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Usually, female patient with narrow AP chest diameter, low body weight, low blood pressure • Mid to late click, heard best at the apex • Crescendo mid to late diastolic murmur • Findings accentuated in the standing position • Most patients with MVP are asymptomatic; symptoms (if present) consist primarily of chest pain and palpitations • Neurologic abnormalities (e.g., TIA or stroke) are rare • Patients may also complain of anxiety, fatigue, and dyspnea

DIAGNOSIS

Echocardiography shows the anterior and posterior leaflets bulging posteriorly in systole.

TREATMENT NONPHARMACOLOGIC THERAPY Avoidance of stimulants (e.g., caffeine, nicotine) in patients with palpitations

ACUTE GENERAL Rx • The empiric use of antiarrhythmic drugs to prevent sudden death in patients with uncomplicated MVP is not advisable; -blockers may be tried in symptomatic patients (e.g., palpitations, chest pain); they decrease the heart rate, thus decreasing the stretch on the prolapsing valve leaflets. • Antibiotic prophylaxis for infective endocarditis when undergoing dental, GI, or GU procedures is indicated only in patients with MVP who have a systolic murmur and echocardiographic evidence of mitral regurgitation.

PEARLS & CONSIDERATIONS COMMENTS • Recent studies suggest that the prevalence of MVP and its propensity to cause symptoms and serious complications have been overestimated in the past. • Asymptomatic patients with MVP and mild or no mitral regurgitation can be evaluated clinically every 3 to 5 yr. High-risk patients should undergo a follow-up examination once a year.

SUGGESTED READINGS Bouknight DP, O’Rourke RA: Current management of mitral valve prolapse, Am Fam Physician 61:3343, 2000. Freed LA: Prevalence and clinical outcome of mitral valve prolapse, N Engl J Med 341:1, 1999. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

CHRONIC Rx

• Myxomatous degeneration of connective tissue of mitral valve • Congenital deformity of mitral valve and supportive structures • Secondary to other disorders (e.g., Ehlers-Danlos, pseudoxanthoma elasticum)

Section I

ETIOLOGY

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BASIC INFORMATION DEFINITION Multiple myeloma is a malignancy of plasma cells characterized by overproduction of intact monoclonal immunoglobulin or free monoclonal kappa or lambda chains. ICD-9CM CODES 203.0 Multiple myeloma

EPIDEMIOLOGY & DEMOGRAPHICS ANNUAL INCIDENCE: 4 cases/100,000 persons (blacks affected twice as frequently as whites); multiple myeloma accounts for 10% of all hematologic cancers PREDOMINANT AGE: Peak incidence in the seventh decade at a median age of 69 yr

PHYSICAL FINDINGS & CLINICAL PRESENTATION The patient usually comes to medical attention because of one or more of the following: • Bone pain (back, thorax) or pathologic fractures caused by osteolytic lesions • Fatigue or weakness because of anemia secondary to bone marrow infiltration with plasma cells • Recurrent infections as a result of impaired neutrophil function and deficiency of normal immunoglobulins • Nausea and vomiting caused by constipation and uremia • Delirium secondary to hypercalcemia • Neurologic complications, such as spinal cord or nerve root compression, blurred vision from hyperviscosity • Pallor and generalized weakness from anemia • Purpura, epistaxis from thrombocytopenia • Evidence of infections from impaired immune system • Bone pain, weight loss • Swelling on ribs, vertebrae, and other bones

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Metastatic carcinoma Lymphoma Bone neoplasms (e.g., sarcoma) Monoclonal gammopathy of undetermined significance (MGUS)

LABORATORY TESTS • Normochromic, normocytic anemia; rouleaux formation on peripheral smear • Hypercalcemia is present in 15% of patients at diagnosis

• Elevated BUN, creatinine, uric acid, and total protein • Proteinuria secondary to overproduction and secretion of free monoclonal kappa or lambda chains (Bence Jones protein) • Tall homogeneous monoclonal spike (M spike) on protein immunoelectrophoresis (IEP) in approximately 75% of patients; decreased levels of normal immunoglobulins 1. The increased immunoglobulins are generally IgG (75%) or IgA (15%). 2. Approximately 17% of patients have flat level of immunoglobulins but increased light chains in the urine by electrophoresis. 3. A very small percentage (2%) of patients have nonsecreting myeloma (no increase in immunoglobulins and no light chains in the urine) but have other evidence of the disease (e.g., positive bone marrow examination). • Reduced ion gap resulting from the positive charge of the M proteins and the frequent presence of hyponatremia in myeloma patients • Hyponatremia, serum hyperviscosity (more common with production of IgA) • Bone marrow examination: usually demonstrates nests or sheets of plasma cells, which comprise 30% of the bone marrow, and 10% are immature • Serum -2 microglobulin has little diagnostic value; it is useful for prognosis because levels 8 mg/L indicate high tumor mass and aggressive disease • Elevated serum levels of LDH at the time of diagnosis define a subgroup of myeloma patients with very poor prognosis • Increased interleukin-6 in serum during active stage of myeloma • The production of DKK1, an inhibitor of osteoblast differentiation, by myeloma cells is associated with the presence of lytic bone lesions in patients with multiple myeloma.

IMAGING STUDIES X-ray films of painful areas may demonstrate punched-out lytic lesions or osteoporosis. Bone scans are not useful, because lesions are not blastic.

TREATMENT NONPHARMACOLOGIC THERAPY Prevention of renal failure with adequate hydration and avoidance of nephrotoxic agents and dye contrast studies

ACUTE GENERAL Rx • Newly diagnosed patients with good performance status are best treated with autologous stem cell transplantation, resulting in improved survival. Useful guidelines (from the Hematology Disease Site Group of the Cancer Care Ontario Practice Guidelines Initiative) regarding the role of high-dose chemotherapy and stem-cell transplantation are as follows: 1. Autologous transplantation is recommended for patients with stage II or III myeloma and good performance status. 2. Allogenic transplantation is not recommended as routine therapy. 3. Patients potentially eligible for transplantation should be referred for assessment early after diagnosis and should not be extensively exposed to alkylating agents before collection of stem cells. 4. Autologous peripheral stem cells should be harvested early in the patient’s treatment course (best when performed as part of initial therapy). 5. A single transplant with high-dose melphalan, with or without total body irradiation, is suggested for patients undergoing transplantation outside a clinical trial. 6. At this time no conclusion can be reached about the role of interferon therapy after transplantation. • Chemotherapeutic agents effective in multiple myeloma are: 1. Melphalan and prednisone: the rates of response to this treatment range from 40% to 60%. Adding continuous low-dose interferon to standard melphalan-prednisone therapy does not improve response rate or survival; however, response duration and plateau phase duration are prolonged by maintenance therapy with interferon. 2. Vincristine, doxorubicin (Adriamycin), and dexamethasone (VAD) can be used in patients not responding or relapsing after treatment with melphalan and prednisone; methylprednisolone is substituted for dexamethasone (VAMP) in some centers. 3. High-dose chemotherapy (HDCT) with vincristine, melphalan, cyclophosphamide, and prednisone (VMCP) alternating with vincristine, carmustine, doxorubicin, and prednisone (BVAP) combined with bone marrow transplantation improves the response rate, event-free survival, and overall survival in patients with myeloma.

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Multiple Myeloma

• Promptly diagnose and treat infections. Common bacterial agents are Streptococcus pneumoniae and Haemophilus influenzae. Prophylactic therapy against Pneumocystis carinii with trimethoprim

• • •

DISPOSITION • Prognosis is better in asymptomatic patients with indolent or smoldering myeloma: median survival time is approximately 10 yr in persons with no lytic bone lesions and a serum myeloma protein concentration 3 g/dl.

• As compared with a single autologous stem-cell transplantation, double transplantation (two successive autologus stem-cell transplantations) improves survival among patients with myeloma, especially those who do not have a very good partial response after undergoing one transplantation.

SUGGESTED READINGS Attal M et al: Single versus double autologous stem-cell transplantation for multiple myeloma, N Engl J Med 349:2495, 2003. Imrie K et al: The role of high dose chemotherapy and stem-cell transplantation in patients with multiple myeloma: a practice guideline of the Cancer Care Ontario Practice Guidelines Initiative, Ann Intern Med 136:619, 2002. Rajkumar SV et al: Current therapy for multiple myeloma, Mayo Clin Proc 77:813, 2002. Tian E et al: The role of WNT-signaling antagonist DKK1 in the development of osteolytic lesions in multiple myeloma, N Engl J Med 349:2483, 2003. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

CHRONIC Rx

•

sulfamethoxazole must be considered in patients receiving chemotherapy and high-dose corticosteroid regimens. Control hypercalcemia and hyperuricemia. Control pain with analgesics; radiation therapy and surgical stabilization may also be indicated. Treat anemia with epoetin alfa. Monthly infusions of the bisphosphonate pamidronate provide significant protection against skeletal complications and improve the quality of life of patients with advanced multiple myeloma. Zoledronic acid (Zometa) can be infused over 15 min and is more effective than pamidronate for treatment of hypercalcemia of malignancy. Bisphosphonates (pamidronate, zoledranate, and ibandronate) also appear to have an antitumor effect.

Section I

4. Current HDCT regimen with autologous stem-cell support achieve complete response in approximately 20% to 30% of patients, with best results seen in good-risk patients, defined as young patients (50 yr of age) with good performance status and a low tumor burden (2 microglobulin 2.5 mg/L). 5. Thalidomide, an agent with antiangiogenic properties, is useful to induce responses in patients with multiple myeloma refractory to chemotherapy. 6. Bortezomib (Velcade) is a newer protease inhibitor that is cytotoxic for multiple myeloma. It is indicated for treatment of refractory multiple myeloma. It is expensive, with an average course of treatment (5 cycles) costing $20,000.

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Myelodysplastic Syndrome BASIC INFORMATION DEFINITION Myelodysplastic syndromes (MDS) are a group of acquired clonal disorders affecting the hemopoietic stem cells and characterized by cytopenias with hypercellular bone marrow and various morphologic abnormalities in the hemopoietic cell lines. MDSs show abnormal (dysplastic) hemopoietic maturation. Marrow cellularity is increased, reflecting an effective hematopoiesis, but inadequate maturation results in peripheral cytopenias. Myelodysplasia encompasses several heterogenous syndromes. The French-AmericanBritish classification of myelodysplastic syndromes includes the following: refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, chronic myelomonocytic leukemia, and refractory anemia with excess blasts in transformation.

SYNONYMS MDS Preleukemia Dysmyelopoietic syndrome ICD-9CM CODES 238.7 Myelodysplastic syndrome

• • • •

Irradiation Autoimmune disease Infections (TB, viral infections) Paroxysmal nocturnal hemoglobinuria

WORKUP • Diagnostic workup includes laboratory evaluation and bone marrow examination. • An algorithmic approach to patients with suspected myelodysplastic syndromes is described in Section III.

LABORATORY TESTS • Anemia with variable MCV (normal or increased) • Reduced reticulocyte count (in relation to the degree of anemia) • Hypogranular or agranular neutrophils • Thrombocytopenia or normal platelet count • Hypogranular platelets may be present • Hypercellular bone marrow, with frequent clonal chromosomal abnormalities

IMAGING STUDIES Abdominal CT scan may reveal hepatosplenomegaly.

TREATMENT

EPIDEMIOLOGY & DEMOGRAPHICS

NONPHARMACOLOGIC THERAPY

INCIDENCE (IN U.S.): Approximately 82 cases/100,000 persons/yr PREDOMINANT AGE: More common in elderly patients, with a median age of 65 yr

RBC transfusions in patients with severe symptomatic anemia

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Splenomegaly, skin pallor, mucosal bleeding, ecchymosis may be present. • Patients often present with fatigue. • Fever, infection, and dyspnea are common.

ETIOLOGY Unknown. However, exposure to radiation, chemotherapeutic agents, benzene, or other organic compounds is associated with myelodysplasia.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Vitamin B12/folate deficiency • Exposure to toxins (drugs, alcohol, chemotherapy) • Renal failure

ACUTE GENERAL Rx • Results of chemotherapy are generally disappointing. • The role of myeloid growth factors (granulocyte colony–stimulating factor, granulocyte-macrophage colony–stimulating factor) and immunotherapy is undefined. In a recent trial, 34% of patients treated with antithymocyte globulin (40 mg/kg for 4 days) became transfusion independent. Response was also associated with a statistically significantly longer survival. • Allogeneic stem-cell transplantation should be considered in patients 60 yr old because this is the established procedure with cure potential.

CHRONIC Rx Monitor for infections, bleeding, and complications of anemia.

DISPOSITION • Cure rates in patients with allogeneic bone marrow transplantations approach 30% to 50%.

• The risk of transformation to acute myelogenous leukemia varies with the percentage of blasts in the bone marrow. • Advanced age, male sex, and deletion of chromosomes 5 and 7 are associated with a poor prognosis. • According to the International Myelodysplastic Syndrome Risk Analysis Workshop, the most important variables in disease outcome are the specific cytogenetic abnormalities, the percentage of blasts in the bone marrow, and the number of hematopoietic lineages involved in the cytopenias.

REFERRAL Hematology referral in all patients with MDS

PEARLS & CONSIDERATIONS COMMENTS • Erythropoietin (epoetin alfa) SC three times weekly may be effective in increasing the Hgb and reducing the RBC transfusion requirement in some patients. • Lenalidomide, a novel analogue of thalidomide, has demonstrated hematologic activity in patients with lowrise myelodysplastic syndrome who have no response to erythropoietin or who are unlikely to benefit from conventional therapy. • Patients with cytogenetic abnormalities associated with poor prognosis should be considered for aggressive treatment with high-dose chemotherapy and stem-cell transplantation. • Nearly 50% of the deaths that result from myelodysplastic syndromes are the result of cytopenia associated with bone marrow failure.

SUGGESTED READINGS List A et al: Efficacy of lenalidomide in myelodysplastic syndromes, N Engl J Med 352:549, 2005. Molldrem JJ et al: Antithymocyte globulin for treatment of the bone marrow failure associated with myelodysplastic syndromes, Ann Intern Med 137:156, 2002. AUTHOR: FRED F. FERRI, M.D.

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Myocardial Infarction

DEFINITION

Clinical presentation: • Crushing substernal chest pain usually lasts longer than 30 min. • Pain is unrelieved by rest or sublingual nitroglycerin or is rapidly recurring. • Pain radiates to the left or right arm, neck, jaw, back, shoulders, or abdomen and is not pleuritic in character. • Pain may be associated with dyspnea, diaphoresis, nausea, or vomiting. • There is no pain in approximately 20% of infarctions (usually in diabetic or elderly patients). Physical findings: • Skin may be diaphoretic, with pallor (because of decreased oxygen). • Rales may be present at the bases of lungs (indicative of CHF). • Cardiac auscultation may reveal an apical systolic murmur caused by mitral regurgitation secondary to papillary muscle dysfunction; S3 or S4 may also be present. • Physical examination may be completely normal.

Acute coronary syndromes are manifestations of ischemic heart disease and represent a broad clinical spectrum that includes unstable angina/non-ST elevation MI and ST-elevation MI. 1. Myocardial infarction is characterized by necrosis resulting from an insufficient supply of oxygenated blood to an area of the heart. According to the joint European Society of Cardiology/ American College of Cardiology, either one of the following criteria for acute evolving or recent MI satisfies the diagnosis: a. Typical rise and gradual fall (troponin) or more rapid rise and fall (CK-MB) of biochemical markers of myocardial necrosis with at least one of the following: i. Ischemic symptoms ii. Development of pathologic Q waves on ECG iii. ECG changes indicative of ischemia (ST-segment elevation or depression) iv. Coronary artery intervention (e.g., coronary angioplasty) b. Pathologic findings of acute MI 2. ST elevation MI: area of ischemic necrosis that penetrates the entire thickness of the ventricular wall and results in ST-segment elevation. 3. Unstable angina: coronary arterial plaque rupture with fragmentation and distal arterial embolization resulting in myocardial necrosis. Usually occurs without ST elevation and is thus termed non-ST elevation MI.

ETIOLOGY • Coronary atherosclerosis • Coronary artery spasm • Coronary embolism (caused by infective endocarditis, rheumatic heart disease, intracavitary thrombus) • Periarteritis and other coronary artery inflammatory diseases • Dissection into coronary arteries (aneurysmal or iatrogenic) • Congenital abnormalities of coronary circulation • Hypercoagulable states, increased blood viscosity (polycythemia vera)

SYNONYMS MI Non-ST elevation MI ST-elevation MI Heart attack Coronary thrombosis Coronary occlusion ICD-9CM CODES 410.9 Acute myocardial infarction, unspecified site

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE/PREVALENCE (IN U.S.): • 500 cases/100,000 persons. • 500,000 MIs in the U.S. yearly. • More prominent in males between the ages of 40 and 65 yr; no predominant sex after age 65 yr. • Women experience more lethal and severe first acute MIs than men, regardless of comorbidity, previous angina, or age. • At least one fourth of all myocardial infections are clinically unrecognized.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS The various causes of myocardial ischemia are described in Section II along with the differential diagnosis of chest pain.

LABORATORY TESTS • Cardiac troponin levels: cardiacspecific troponin T (cTnT) and cardiacspecific troponin I (cTnl) are highly specific for myocardial injury. Increases in serum levels of cTnT and cTnI may occur relatively early after muscle damage (3-12 hr), peak within 24 hr, and may be present for several days after MI (up to 7 days for cTnI and up to 10-14 days for cTnT). Troponin T tests can be falsely positive in patients with renal failure. The threshold level of troponin T considered positive for MI is 0.1 ng/ml in patients with normal renal function or 0.5 ng/ml in patients with renal impairment.

• Creatine kinase MB isoenzyme is a useful marker for MI. It is released in the circulation in amounts that correlate with the size of the infarct. • Neither CK-MB nor troponin consistently appears in the blood within 6 hr after an ischemic event; therefore serial testing (e.g., on presentation and after 8 hr) is necessary to definitely rule out MI. • ECG: 1. In ST-elevation MI, there is development of: a. Inverted T waves, indicating an area of ischemia b. Elevated ST segment, indicating an area of injury c. Q waves, indicating an area of infarction (usually develop over 12 to 36 hr) 2. In unstable angina/non-ST elevation MI, Q waves are absent, but the following indications are present: a. History and myocardial enzyme elevations are compatible with MI. b. ECG shows ST segment elevation, depression, or no change followed by T wave inversion.

IMAGING STUDIES • Chest radiography is useful to evaluate for pulmonary congestion and exclude other causes of chest pain. • Echocardiography can evaluate wall motion abnormalities and identify mural thrombus or mitral regurgitation, which can occur acutely after MI.

TREATMENT NONPHARMACOLOGIC THERAPY • Limit patient’s activity: bed rest for the initial 24 hr; if the patient remains stable, gradually increase activity. • Diet: NPO until stable, then no added salt and a low-cholesterol diet. • Patient education to decrease the risk of subsequent cardiac events (proper diet, cessation of smoking, regular exercise) should be initiated when the patient is medically stable.

ACUTE GENERAL Rx • Any patient with suspected acute MI should immediately receive the following: 1. Aspirin: give 160 to 325 mg PO unless true aspirin allergy is suspected. If the first dose is chewed, a blood level is achieved more rapidly than if it is swallowed. Clopidogrel may be substituted if true allergy is present. 2. Nitrates: they increase the supply of oxygen by reducing coronary vasospasm and decrease consumption of oxygen by reducing

DISEASES AND DISORDERS

PHYSICAL FINDINGS & CLINICAL PRESENTATION

Section I

BASIC INFORMATION

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Myocardial Infarction ventricular preload. Sublingual nitroglycerin can be administered immediately on suspicion of MI (unless systolic blood pressure is 90 mm Hg or heart rate is 50 bpm or 100 bpm); IV nitroglycerin can be subsequently used. Nitroglycerin should be used with great caution in patients with inferior wall MI; nitrate usage can result in hypotension because these patients are sensitive to change in preload. It should also be avoided in patients suspected of having right ventricular infarction (increased risk of preload reduction) and if a patient has used sildenafil (Viagra), or vardenafil (Levitra) within the previous 24 hr or tadalafil (Cialis) in the previous 36 to 48 hr. 3. Adequate analgesia: morphine sulfate 2 mg IV q5min PRN can be given for severe pain unrelieved by nitroglycerin. Hypotension secondary to morphine can be treated with careful IV hydration with saline solution. If sinus bradycardia accompanies hypotension, use atropine (0.5 to 1.0 mg IV q 5 min PRN to a total dose of 2.5 mg). Respiratory depression caused by morphine can be reversed with naloxone (Narcan) 0.8 mg. 4. Nasal oxygen: administer at 2 to 4 L/min. • If readily available without delay, percutaneous coronary intervention (PCI) with adjunctive glycoprotein IIb/IIIa is preferred over thrombolytic therapy. It is effective and generally results in more favorable outcomes than thrombolytic therapy. When PCI is performed, use of IV heparin is recommended. Coronary stents are useful to decrease ischemia, improve long-term patency, and lower the rate of restenosis of the infarct-related artery. • Thrombolytic therapy: if the duration of pain has been 6 hr and primary angioplasty is not readily available, recanalization of the occluded arteries should be attempted with thrombolytic agents, possibly in combination with glycoprotein IIb/IIIa inhibition. Because the effectiveness of thrombolytics is time-dependent, ideally these agents should be administered either in the field or within 30 min of the patient’s arrival in the emergency department. When tPA or rPA is used, IV heparin is given to increase the likelihood of patency in the infarct-related artery. In patients receiving streptokinase or APSAC, IV heparin is not indicated, because it does not offer any additional benefit and can result in increased bleeding complications. Tenecteplase and reteplase are comparable with accelerated infusion recombinant TPA in terms of

efficacy and safety, but are more convenient because they are administered by bolus injection. Lanoplase and heparin bolus plus infusion is as effective as TPA with regard to mortality, but the rate of intracranial hemorrhage is significantly higher. Absolute contraindications to thrombolytic therapy include active internal bleeding, intracranial neoplasm or arteriovenous malformation, intracranial surgery in past 6 mo, stroke in past year, head trauma with loss of consciousness in past 6 mo, surgery in noncompressible location in past 6 wk, alteration in mental status, and infectious endocarditis. • -adrenergic blocking agents should be given to all patients with evolving acute MI, provided that there are no contraindications (see below). -Blockers are useful to reduce myocardial oxygen consumption and prevent tachyarrhythmias. Early IV blockage (in the initial 24 hr) followed by institution of an oral maintenance regimen is also effective in reducing recurrent infarction and ischemia. Frequently used agents are: 1. Metoprolol (Lopressor): IV 5 mg q2min 3 doses, then PO 25 to 50 mg q6h, given 15 min after last IV dose, continued for 48 hr; maintenance dosage is 50 to 100 mg bid. 2. Atenolol (Tenormin): IV 5 mg over 5 min, repeat in 10 min if initial dose is well tolerated, then start PO dose 10 min after the last IV dose; PO 50 mg qd, increasing to 100 mg as tolerated. Before using -blockers, some of the contraindications and side effects (i.e., exacerbation of asthma, CNS effects, hypertension, bradycardia) must be carefully assessed. • ACE inhibitors reduce left ventricular dysfunction and dilation and slow the progression of CHF during and after acute MI. They should be initiated within hours of hospitalization, provided that the patient does not have hypotension or a contraindication (bilateral renal stenosis, renal failure, or history of angioedema caused by previous treatment with ACE inhibitors). 1. Commonly used agents are captopril 12.5 mg PO bid, enalapril 2.5 mg bid, or lisinopril 2.5 to 5 mg qd initially, with subsequent titration as needed. 2. ACE inhibitors may be stopped in patients without complications and no evidence of left ventricular dysfunction after 6 to 8 wk. 3. ACE inhibitors should be continued indefinitely in patients with impaired left ventricular function (ejection fraction 40%) or clinical CHF. • Glycoprotein IIb receptor inhibitors (tirofiban, eptifibatide), when adminis-

tered with heparin and aspirin, further reduce the incidence of ischemic events in patients with non-Q wave MI. The use of IV glycoprotein IIb/IIIa inhibitors (e.g., abciximab) before and during PTCA also reduces the risk of closure postangioplasty. • Initiation of statin therapy before hospital discharge.

CHRONIC Rx Discharge medications in all patients with MI/NSTEMI (unless contraindicated) should include antiischemic medications (e.g., nitroglycerin, -blocker), lipidlowering agents, and aspirin (81-325 mg/day). Clopidogrel (Plavix) 75 mg/day can be given in addition to aspirin for up to nine months or in place of aspirin in those who cannot tolerate aspirin. The addition of ACE inhibitors is also recommended in all patients with diabetes, CHF, and in those with EF 40%. Evaluation of post-MI patients • Submaximal (low level) treadmill test (can be done 1 to 3 wk after MI) in stable patients without any clinical evidence of significant left ventricular dysfunction or post-MI angina 1. Useful to assess the patient’s functional capacity and formulate an athome exercise program 2. Helpful to determine the patient’s prognosis • Radionuclide angiography or twodimensional echocardiography 1. To evaluate patient’s left ventricular ejection fraction 2. To evaluate ventricular size and segmental wall motion 3. Echocardiography to rule out presence of mural thrombi in patients with anterior wall infarction; transesophageal echo is preferred if mural thrombosis is suspected • A 24-hr Holter monitor study to evaluate patients who have demonstrated significant arrhythmias during their hospital stay; selected patients with complex ventricular ectopy may be candidates for programmed electrical stimulation studies and antiarrhythmic therapy and/or implanted defibrillator, depending on the results of these studies

DISPOSITION The prognosis after MI depends on multiple factors: • Use of -blockers: the mortality of patients on a regular regimen of -blockers is significantly decreased when compared with that of control groups. Discharge medication in patients with MI/NSTEMI should include a -blocker in all patients without contraindications. • Presence of arrhythmias, frequent ventricular ectopy (10/hr), or repetitive

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Myocardial Infarction

• • •

• •

•

SUGGESTED READINGS Andersen HR et al: A comparison of coronary angioplasty with fibrinolytic therapy in acute myocardial infarction, N Engl J Med 349:733, 2003. Becker RC: Antithrombotic therapy after myocardial infarction, N Engl J Med 347:1019, 2002. Birnbaum Y et al: Ventricular septal rupture after acute myocardial infarction, N Engl J Med 347:1426, 2002. Brennan ML et al: Prognostic value of myeloperoxidase in patients with chest pain. N Engl J Med 349:1595, 2003. Cannon CP, Baim DS: Expanding the reach of primary percutaneous coronary intervention for the treatment of acute myocardial infarction, J Am Coll Cardiol 39:1720, 2002.

Cannon CP et al: Intensive versus moderate lipid lowering with statins after acute coronary syndromes, N Engl J Med 350:1495, 2004. Dickstein K et al: Effects of losartan and captopril on mortality and morbidity in highrisk patients after acute myocardial infarction: the OPTIMAAL randomized trial, Lancet 360:752, 2002. Hurlen et al: Warfarin, aspirin, or both after myocardial infarction, N Engl J Med 347:969, 2002. Khot UN et al: Prognostic importance of physical examination for heart failure in Non-ST elevation acute coronary syndromes, JAMA 290:2174, 2003. Meier MA et al: The new definition of myocardial infarction, Arch Intern Med 162: 1585, 2002. Moss AJ et al: Prophylactic implantation of a defibrillator in patients with myocardial infarction and reduced ejection fraction, N Engl J Med 346:877, 2002. Newby LK et al: Early statin initiation and outcomes in patients with acute coronary syndromes, JAMA 287:3087, 2002. Stenestrand U, Wallentin L: Early revascularisation and 1-year survival in 14-day survivors of acute myocardial infarction: a prospective cohort study, Lancet 359:1805, 2002. Stone GW et al: Comparison of angioplasty with stenting, with or without abciximab, in acute myocardial infarction, N Engl J Med 346:957, 2002. Wiviott SD, Braunwauld E: Unstable Angina and Non-ST-Segment Elevation Myocardial Infarction, Am Fam Physician 70:525, 2004. Zimetbaum PJ, Josephson ME: Use of electrocardiogram in acute myocardial infarction, N Engl J Med 348:933, 2003. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

•

• Self-reported moderate alcohol consumption in the year before acute MI is associated with reduced 1-yr mortality. • Discharge medication in patients with MI/NSTEMI should include lipid-lowering agents in patients with hyperlipidemia unresponsive to exercise and dietary restrictions. Statins may also lower vascular inflammation and damage by mechanisms other than reduction of LDL cholesterol. Early initiation of statin treatment in patients with acute MI is associated with reduced 1-yr mortality. • Additional poor prognostic factors include the following: cigarette smoking, history of hypertension or prior MI, presence of ST segment depression in acute MI, increasing age, diabetes mellitus, and female sex.

Section I

•

forms of ventricular ectopic beats (couplets, triplets) indicates an increased risk (two to three times greater) of sudden cardiac death. New bundle branch block, Mobitz II second-degree block, and third-degree heart block also adversely affect outcome. Size of infarct: the larger it is, the higher the post-MI mortality rate. Significant myocardial stunning with subsequent improvement of ventricular function occurs in most patients after anterior MI. A lower level of creatine kinase, an estimate of the extent of necrosis, is independently predictive of recovery of function. Site of infarct: inferior wall MI carries a better prognosis than anterior wall MI; however, patients with inferior wall MI and right ventricular involvement have a high risk for arrhythmic complications and cardiac shock. Ejection fraction after MI: the lower the left ventricular ejection fraction, the higher the mortality after MI. Presence of post-MI angina indicates a high mortality rate. Performance on low-level exercise test: the presence of ST segment changes during the test is a predictor of high mortality during the first year. Presence of pericarditis during the acute phase of MI increases mortality at 1 yr. Type A behavior (competitive drive, ambitiousness, hostility) is associated with a lower mortality rate after symptomatic MI. The Killip classification is an independent predictor of all-cause mortality in patients with non-ST elevation acute coronary syndromes.

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Nonalcoholic Fatty Liver Disease BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Liver disease occurring in patients who do not abuse alcohol and manifested histologically by mononuclear cells and/or polymorphonuclear cells, hepatocyte ballooning, and spotty necrosis.

• Alcohol-induced liver disease (a daily alcohol intake of 20 g in females and 30 g in males [three 12-oz beers or 12 oz of wine] may be enough to cause alcohol-induced liver disease) • Viral hepatitis • Autoimmune hepatitis • Toxin or drug-induced liver disease

SYNONYMS • Nonalcoholic steatohepatitis (NASH) • Fatty liver hepatitis • Diabetes hepatitis ICD-9CM CODES 571.8 Fatty liver

EPIDEMIOLOGY & DEMOGRAPHICS • Nonalcoholic fatty liver disease affects 10% to 24% of general population • Increased prevalence in obese persons (57% to 74%), type 2 diabetes mellitus, and hyperlipidemia (primarily hypertriglyceridemia) • Most common cause of abnormal liver test results in adults in the U.S. (accounts for up to 90% of cases of asymptomatic ALT elevations) • 30 million obese adults have steatosis, 8.6 million may have steatohepatitis

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Most patients are asymptomatic • Patients may report a sensation of fullness or discomfort on the right side of the upper abdomen • Nonspecific complaints of fatigue or malaise may be reported • Hepatomegaly is generally the only positive finding on physical examination

ETIOLOGY • Insulin resistance is the most reproducible factor in the development of nonalcoholic fatty liver disease • Risk factors are obesity (especially truncal obesity), diabetes mellitus, hyperlipidemia

WORKUP Diagnosis is usually suspected on the basis of hepatomegaly, asymptomatic elevations of transaminases, or “fatty liver” on sonogram of abdomen in obese patients with little or no alcohol use. Liver biopsy will confirm diagnosis and provide prognostic information. It should be considered in patients with suspected advanced liver fibrosis (presence of obesity or type 2 diabetes, AST/ALT ratio 1).

LABORATORY TESTS • Elevated ALT, AST: AST/ALT ratio is usually 1, but can increase as fibrosis advances • Negative serology for infectious hepatitis; generally normal GGTP, and serum alkaline phosphatase • Hyperlipidemia (primarily hypertriglyceridemia) may be present • Elevated glucose levels may be present • Prolonged prothrombin time, hypoalbuminuria, and elevated bilirubin may be present in advanced stages • Elevated serum ferritin and increased transferrin saturation may be found in up to 10% of patients; however, hepatic iron index and hepatic iron level are normal • Liver biopsy may show a wide spectrum of liver damage, ranging from simple steatosis to advanced fibrosis and cirrhosis

IMAGING STUDIES • Ultrasound generally reveals diffuse increase in echogenicity as compared with that of the kidneys; CT scan reveals diffuse low-density hepatic parenchyma.

• Occasionally patients may have focal rather than diffuse steatosis, which may be misinterpreted as a liver mass on ultrasound or CT; use of MRI in these cases will identify focal fatty infiltration.

TREATMENT NONPHARMACOLOGIC THERAPY Weight reduction in all obese patients. Exercise is also effective.

GENERAL THERAPY • No medications have been proved to directly improve liver damage from nonalcoholic fatty liver disease. • Medications to control hyperlipidemia (e.g., fenofibrates for elevated triglycerides) and hyperglycemia (e.g., metformin) can lead to improvement in abnormal liver test results. Metformin, pioglitazone, and rosiglitazone may be effective but are not yet FDA-aproved for this purpose.

DISPOSITION • Patients with pure steatosis on liver biopsy generally have a relatively benign course. • The presence of steatohepatitis or advanced fibrosis on liver biopsy is associated with a worse prognosis.

REFERRAL Liver transplantation should be considered in patients with decompensated, end-stage disease; however, in these patients there may be a recurrence of nonalcoholic fatty liver disease posttransplantation.

SUGGESTED READINGS Angulo P: Nonalcoholic fatty liver disease, N Engl J Med 346:1221, 2002. Clark JM: Nonalcoholic fatty liver disease, JAMA 289:3000, 2003. Dixon JB et al: Nonalcoholic fatty liver disease: predictors of nonalcoholic steatohepatitis and liver fibrosis in the severely obese, Gastroenterology 121:91, 2001. AUTHOR: FRED F. FERRI, M.D.

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Obesity BASIC INFORMATION

SYNONYMS Overweight ICD-9CM CODES 278.0 Obesity

EPIDEMIOLOGY & DEMOGRAPHICS • Approximately 97 million adults in the U.S. and 310 million people worldwide are overweight or obese. • The present costs of obesity in the U.S. population are estimated to run at 5%8% of total healthcare spending, which equates to $92.6-$99.2 billion annually (1998 data normalized to 2002 dollars). • From 1960 to 1999, the prevalence of excess weight (BMI 25 kg/m2) increased from 44% to 61% of the adult population, and the prevalence of obesity (BMI 30 kg/m2) doubled, from 13% to 27%. Estimates in 2003 suggest that 31% of the U.S. population is now obese. • In the U.S., the progression of obesity is 3-4 yr ahead of the problem in Europe. • Overweight and obesity are defined as stated previously on the basis of epidemiologic data showing increased mortality with BMIs above 25 kg/m2. • For persons with a BMI of 30 kg/m2, all-cause mortality is increased by 50% to 100% above that of persons with BMIs in the range of 20 to 25 kg/m2. • Obese individuals are at increased risk of morbidity/mortality from type 2 diabetes, hypertension, CVD, cancer (particularly breast cancer), sleep apnea, osteoarthritis, and skin disorders. • The effects of obesity on health outcome appear to be reversible with weight loss. • Obesity is more prevalent in black and Hispanic women compared with nonHispanic white women and men. Approximately 50% of the black female population is estimated to be obese. • People in the U.S. with low incomes or low education are 5% more likely to be obese than those of higher socioeconomic status. • In 1993 the Deputy Assistant Secretary for Health (J. Michael McGinnis) and the former Director of the Centers for Disease Control and Prevention (CDC) (William Foege) coauthored a journal article, “Actual Causes of Death in the

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Obesity is self-evident on examination. • Measuring the height in meters and weight in kilograms determines your BMI. • Increased waist circumference (40 inches in men and 35 inches in women) is apparent. • Hypertension is related to obesity. • Symptoms of diabetes (e.g., polyuria, polydipsia, retinopathy, and neuropathy) may be present. • Joint pain and swelling are associated with osteoarthritis and obesity. • Dyspnea may be present.

ETIOLOGY • The cause of obesity is multifactorial, involving social, cultural, behavioral, physiologic, metabolic, and genetic factors. • Supporting genetic factors come from identical twins reared apart and “obesity genes” encoding for the appetitesuppressant hormone leptin. • Environmental factors are a major determinant of obesity with the underlying theme of excess calorie intake and lack of physical activity. • Genetics and environmental factors exert their effects on energy balance and obesity via effects on behavior and physiology. There is no direct link between genetics and body weight or obesity. Obesity develops as a result of excessive energy intake, inadequate energy expenditure, or both. •Over the last 2 decades, fat consumption has declined in parallel with the increased prevalence of obesity in both the U.S. and Europe, and the decline is matched by a parallel increase in carbohydrate consumption, suggesting a role for excessive dietary carbohydrate in the development of obesity.

DIAGNOSIS • Determination of the BMI establishes the diagnosis of obesity according to the previous definition and assesses the individual’s risk for disease. • BMI is defined as the weight in kilograms divided by the square of the height in meters (WH2). • Strict BMI measurements should be used with caution in making a diagnosis of obesity.

It is important to rule out specific causative medical disorders in obese patients. Hypothalamic disorders, hypothyroidism, Cushing’s syndrome, insulinoma, and chronic corticosteroid use can cause obesity.

WORKUP The workup of an obese patient typically requires laboratory work to assess for risks and complications as well as to rule out underlying causative medical conditions.

LABORATORY TESTS • Laboratory tests are not specific in diagnosing obesity; however, they are used to identify diabetes and hyperlipidemia commonly related to excess weight. • In the proper clinical setting, thyroid function studies (TSH, free T4), AM cortisol level, and insulin level with Cpeptide measurements will exclude hypothyroidism, Cushing’s syndrome, and insulinoma as underlying causes of obesity.

IMAGING STUDIES • X-ray imaging studies are not specific in the diagnosis of obesity. • Several methods are available for determining or calculating total body fat but offer no significant advantage over the BMI. 1. Total body water 2. Total body potassium 3. Bioelectrical impedance 4. Dual-energy x-ray absorptiometry • Buoyancy testing is the most accurate method for determining total body fat composition.

TREATMENT • Treatment is aimed at weight reduction and risk factor modification (e.g., diabetes, lipids, hypertension). • Once a joint decision between patient and clinician has been made to lose weight, the expert panel recommends as an initial goal the loss of 10% of baseline weight, to be lost at a rate of 1 to 2 lb/wk over a 6- to 12-mo period followed by long-term maintenance of reduced weight.

NONPHARMACOLOGIC THERAPY • The three major components of weight loss therapy are: 1. Many studies demonstrate that obese adults can lose about 0.5 kg per wk by decreasing their daily intake to 500 to 1000 kcal below the caloric intake required for the maintenance of their current weight.

DISEASES AND DISORDERS

Obesity refers to excess body fat defined as a body mass index (BMI) 30 kg/m2. Overweight is defined as BMI of 25 to 29.9 kg/m2. These conditions result from a problem of imbalance between energy intake and expenditure.

DIFFERENTIAL DIAGNOSIS Section I

DEFINITION

U.S.” It concluded that a combination of dietary factors and sedentary activity patterns accounts for at least 300,000 deaths each year, and obesity is the second leading cause of preventable death in the United States.

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Obesity 2. Increased physical activity initially by walking 30 min 3 times/wk and gradually build up to intense walking 45 min 5 days/wk. The eventual goal is at least 30 min of moderate intense walking. 3. Behavioral therapy is also necessary.

ACUTE GENERAL Rx • Medications for the treatment of obesity are currently approved as an adjunct to diet and physical activity for patients with a BMI of 30 with no concomitant obesity-related risk factors or diseases, and for patients with a BMI 27 with concomitant obesityrelated risk factors or diseases. • Medications approved for the treatment of obesity include 1. Sibutramine 5-15 mg/day 2. Orlistat 120 mg 3 times/day with or within 1 hour after fat-containing meals, plus a daily vitamin. • Only sibutramine and orlistat are approved for long-term use. The safety and efficacy of weight loss medications beyond 2 yr of use have not been established, nor have they been established for the geriatric population. • Medications are divided into appetite suppressants (e.g., sibutramine) and those that decrease nutrient absorption (e.g., orlistat). • Side effects of sibutramine include increases in blood pressure and pulse, dry mouth, headache, insomnia, and constipation. Side effects of orlistat include oily spotting, flatus with discharge, and fecal urgency. • Other medications in clinical trials include bupropion (Wellbutrin), topiramate (Topamax), and metformin (Glucophage).

CHRONIC Rx • Bariatric surgery is a consideration in clinically severe obesity (e.g., BMI  40 or  35 with comorbid conditions). • Gastroplasty, gastric banding, gastric partitioning, and gastric bypass are the surgical procedures performed.

DISPOSITION • Obesity increases the risk of developing hypertension, hyperlipidemia, type 2 diabetes, coronary artery disease, cerebrovascular disease, osteoarthritis, sleep apnea, and endometrial, breast, prostate, and colon cancers. • All-cause mortality is increased in obese patients.

REFERRAL Obesity is commonly seen in the primary care setting. If pharmacologic therapy is considered, consultation with physicians specializing in obesity and experienced with the use of the drug is recommended. In addition, consultation with nutritionists and behavioral therapists is helpful. A consultation with general surgery is indicated in patients being considered for surgical intervention.

PEARLS & CONSIDERATIONS COMMENTS • The National Heart, Lung, and Blood Institute’s (NHLBI) Obesity Education Initiative in cooperation with the National Institute of Diabetes convened the Expert Panel on the Identification, Evaluation, and Treatment of Overweight and Obesity in Adults in May 1995 and have since published evidence-based clinical guidelines for treatment of obesity.

• Only about 20% of adult men and women actually restrict caloric intake and increase physical activity to consciously control body weight. • As knowledge of the physiologic process governing maintenance of body weight increases, newer drug therapies are emerging, which will target lipid metabolic enzymes involved in digestion, absorption, synthesis, storage, and mobilization of fat within the human body

SUGGESTED READINGS Blanck HM et al: Use of non-prescription weight loss products: results from a multistate survey, JAMA 286:930, 2001. Clinical Guidelines on the Identification, Evaluation, and Treatment of Overweight and Obesity in Adults. The Evidence Report. National Institute of Health, National Heart, Lung, and Blood Institute. www.nhlbi.nih. gov/guidelines/obesity/ob_gdlns.pdf Executive Summary of the Clinical Guidelines on the Identification, Evaluation, and Treatment of Overweight and Obesity in Adults, Arch Intern Med 158(17):1855, 1867, 1998. Korner J, Aronne LJ: Pharmacological approaches to weight reduction: Therapeutic targets, J Clin End Metab 89(6)2616, 2004. Lyznicki JM et al: Obesity: assessment and management in primary care, Am Fam Physician 63:2185, 2001. McTigue KM et al: Screening and interventions for obesity in adults: summary of the evidence for the U.S. Preventive Services Task Force, Ann Intern Med 139:933, 2003. Speakman JR: Obesity: the integrated roles of environment and genetics, J Nutr 134:2090S, 2004. Weil E et al: Obesity among adults with disabling conditions, JAMA 288:1265, 2002. Wilson PW et al: Overweight and obesity as determinants of cardiovascular risk, Arch Intern Med 162:1867, 2002. Yanovski SZ, Yanovski JA: Obesity: drug therapy, N Engl J Med 346(8):591, 2002. AUTHORS: JASON IANNUCCILLI, M.D., and PETER PETROPOULOS, M.D.

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Onychomycosis BASIC INFORMATION Onychomycosis is defined as a persistent fungal infection affecting the toenails and fingernails. Tinea unguium Ringworm of the nails ICD-9CM CODES 110.1 Onychomycosis

EPIDEMIOLOGY & DEMOGRAPHICS • Onychomycosis is most commonly found in people above age 40. • Onychomycosis rarely occurs before puberty. • Incidence: 20 to 100 cases/1000 population. • Toenail infection is four to six times more common than fingernail infections. • Onychomycosis affects men more often than women. • Occurs more frequently in patients with diabetes, peripheral vascular disease, and any conditions resulting in the suppression of the immune system. • Occlusive footwear, physical exercise followed by communal showering, and incompletely drying the feet predisposes the individual to developing onychomycosis.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Onychomycosis causes nails to become thick, brittle, hard, distorted, and discolored (yellow to brown color). Eventually, the nail may loosen, separate from the nail bed, and fall off (Fig. 1-24).

• The most common causes of onychomycosis are dermatophyte, yeast, and nondermatophyte molds. • The dermatophyte Trichophyton rubrum accounts for 80% of all nail infections caused by fungus. • Trichophyton interdigitale and Trichophyton mentagrophytes are other fungi causing onychomycosis. • The yeast Candida albicans is responsible for 5% of the cases of onychomycosis. • Nondermatophyte molds Scopulariopsis brevicaulis and Aspergillus niger, although rare, can also cause onychomycosis. • Onychomycosis is classified according to the clinical pattern of nail bed involvement. The main types are: 1. Distal and lateral subungual onychomycosis (DLSO) 2. Superficial onychomycosis 3. Proximal subungual onychomycosis 4. Endonyx onychomycosis 5. Total dystrophic onychomycosis

DIAGNOSIS The diagnosis of onychomycosis is based on the clinical nail findings and confirmed by direct microscopy and culture.

DIFFERENTIAL DIAGNOSIS • • • • • • • • •

Psoriasis Contact dermatitis Lichen planus Subungual keratosis Paronychia Infection (e.g., Pseudomonas) Trauma Peripheral vascular disease Yellow nail syndrome

The workup of suspected onychomycosis is directed at confirming the diagnosis of onychomycosis by visualizing hyphae under the microscope or by growing the organism in culture.

LABORATORY TESTS • Blood tests are not specific in the diagnosis of onychomycosis • KOH prep • Fungal cultures on Sabouraud medium

IMAGING STUDIES • Imaging studies are not very specific in making the diagnosis of onychomycosis. • If an infection is present and osteomyelitis is a consideration, an x-ray of the specific area and a bone scan may help establish the diagnosis.

TREATMENT NONPHARMACOLOGIC THERAPY • Surgical removal of the nail plate is a treatment option; however, the relapse rate is high. • Prevention of reinfection by wearing properly fitted shoes, avoiding public showers, and keeping feet and nails clean and dry.

ACUTE GENERAL Rx • Topical antifungal creams are used for early superficial nail infections. 1. Miconazole 2% cream applied over the nail plate bid 2. Clotrimazole 1% cream bid • Oral agents 1. Itraconazole a. For toenails: 200 mg qd  3 mo b. For fingernails: 200 mg PO bid  7 days, followed by 3 wk of no medicine, for two pulses

A

B

FIGURE 1-24 A, Superficial white onychomycosis. B, Distal subungual onychomycosis. (From Noble J [ed]: Textbook of primary care medicine, ed 3, St Louis, 2002, Mosby.)

DISEASES AND DISORDERS

SYNONYMS

ETIOLOGY

WORKUP Section I

DEFINITION

• Onychomycosis is frequently associated with tinea pedis (athlete’s foot).

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Onychomycosis

• •

• •

2. Terbinafine a. For toenails: 250 mg/day for 3 mo b. For fingernails: 250 mg/day for 6 wk 3. Fluconazole a. For toenails: 150 to 300 mg once weekly, until infection clears b. For fingernails: 150 to 300 mg once weekly until infection clears All oral agents used for onychomycosis require periodic monitoring of liver function blood tests. Itraconazole is contraindicated in patients taking cisapride, astemizole, triazolam, midazolam, and terfenadine. Statins should be discontinued during itraconazole therapy. Fluconazole is contraindicated in patients taking cisapride and terfenadine. Ciclopirox, a topical nail lacquer antifungal agent, is FDA approved for treatment of mild to moderate disease not involving the lunula.

CHRONIC Rx See under “Acute General Rx.”

DISPOSITION • Spontaneous remission of onychomycosis is rare. • A disease-free toenail is reported to occur in approximately 25% to 50% of patients treated with the oral antifungal agents mentioned previously.

REFERRAL • Podiatry consultation is indicated in diabetic patients for proper instruction in foot care, footwear, and nail debridement or surgical removal of the toenail. • Dermatology consultation is indicated in patients refractory to treatment or if another diagnosis is considered (e.g., psoriasis).

PEARLS & CONSIDERATIONS COMMENTS • The growth of fungus on an infected nail typically begins at the end of the nail and spreads under the nail plate to infect the nail bed as well.

• Please review informational insert regarding drug-drug interactions and contraindications before initiating oral antifungal agents.

SUGGESTED READINGS Elewski BE, Hay RJ: Update on the management of onychomycosis: highlights of the Third International Summit on Cutaneous Antifungal Therapy, Clin Infect Dis 23:305, 1996. Epstein E: How often does oral treatment of toenail onychomycosis produce a diseasefree nail? an analysis of published data, Arch Dermatol 134(12):1551, 1998. Gupta AK: The new oral antifungal agents for onychomycosis of the toenails, J Eur Acad Dermatol Venereol 13(1):1, 1999. Rodgers P, Bassler M: Treating onychomycosis, Am Fam Physician 63:663, 2001. Scher RK, Coppa LM: Advances in the diagnosis and treatment of onychomycosis, Hosp Med 34(4):11, 1998. AUTHOR: DENNIS MIKOLICH, M.D.

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Osteoarthritis DISPOSITION

DEFINITION

DIFFERENTIAL DIAGNOSIS

Osteoarthritis is a joint condition in which degeneration and loss of articular cartilage occur, leading to pain and deformity. Two forms are usually recognized: primary (idiopathic) and secondary. The primary form may be localized or generalized.

• • • •

Progression is not always inevitable, and the prognosis is variable depending on the site and extent of the disease.

SYNONYMS Degenerative joint disease Osteoarthrosis Arthrosis ICD-9CM CODES 715.0 Osteoarthrosis and allied disorders

Bursitis, tendinitis Radicular spine pain Inflammatory arthritides Infectious arthritis

• No diagnostic test exists for degenerative joint disease. • Laboratory evaluation is normal. • Rheumatoid factor, ESR, CBC, and ANA tests may be required if inflammatory component is present. • Synovial fluid examination is generally normal.

IMAGING STUDIES

PREVALENCE: 2% to 6% of general population PREDOMINANT SEX: Female  male PREDOMINANT AGE: 50 yr

PHYSICAL FINDINGS & CLINICAL PRESENTATION

TREATMENT

• Similar symptoms in most forms: stiffness, pain, and crepitus • Joint tenderness, swelling • Decreased range of motion • Crepitus with motion • Bony hypertrophy • Pain with range of motion • DIP joint involvement possibly leading to development of nodular swellings called Heberden’s nodes (Fig. 1-25) • PIP joint involvement possibly leading to development of nodular swellings called Bouchard’s nodes

ACUTE GENERAL Rx

ETIOLOGY

• Mild analgesics for joint pain • NSAIDs if inflammation is present • Occasional local corticosteroid injections • Viscosupplementation (injection of hyaluronic acid products into the degenerative joint) is of uncertain benefit • Nutritional supplements (glucosamine and chondroitin) are unproven

Primary osteoarthritis is of unknown cause. Secondary osteoarthritis may result from a number of disorders including trauma, metabolic conditions, and other forms of arthritis.

Surgical consultation for patients not responding to medical management

WORKUP

• Roentgenographic evaluation reveals: 1. Joint space narrowing 2. Subchondral sclerosis 3. New bone formation in the form of osteophytes • When knee is involved, standing AP x-ray

EPIDEMIOLOGY & DEMOGRAPHICS

REFERRAL

• Rest, restricted use or weight bearing, and heat • Walking aids such as a cane (often helpful for weight-bearing joints) • Suitable footwear • Gentle range of motion and strengthening exercise • Local creams and liniments to provide a counterirritant effect • Education, reassurance

PHARMACOLOGIC THERAPY

FIGURE 1-25 Osteoarthritis of the distal interphalangeal (DIP) joints. This patient has the typical clinical findings of advanced osteoarthritis of the DIP joints, including large, firm swellings (Heberden’s nodes), some of which are tender and red because of associated inflammation of the periarticular tissues as well as of the joint. (From Klippel J, Dieppe P, Ferri F [eds]: Primary care rheumatology, London, 1999, Mosby.)

PEARLS & CONSIDERATIONS COMMENTS Surgical intervention is generally helpful in degenerative joint disease. Arthroplasty, arthrodesis, and realignment osteotomy are the most common procedures performed. Arthroscopic debridement (of the knee) appears to be of only limited value.

SUGGESTED READINGS Callahan JJ et al: Results of Charnley total hip arthroplasty at a minimum of thirty years, J Bone Joint Surg 86A:690, 2004. Felson DT: Hyaluronate sodium injections for osteoarthritis: hope, hype and hard truths, Arch Intern Med 162:245, 2002. Hartofilakidis G, Karachalios T: Idiopathic osteoarthritis of the hip: incidence, classification and natural history of 272 cases, Orthopedics 26:161, 2003. Hinton R et al: Osteoarthritis: diagnosis and therapeutic considerations, Am Fam Physician 65:841, 2002. Hunt SA, Jazrawi LM, Sherman OH: Arthroscopic management of osteoarthritis of the knee, J Am Orthop Surg 10:356, 2002. Kelly MA et al: Osteoarthritis and beyond: a consensus on the past, present and future of hyaluronans in orthopedics, Orthopedics 26:1064, 2003. Leopold S et al: Corticosteroid compared with hyaluronic acid injections for the treatment of osteoarthritis of the knee, J Bone Joint Surg 85:1197, 2003. Lo HG: Intra-articular hyaluronic acid in treatment of knee osteoarthritis, JAMA 290:3115, 2003. Moseley JB et al: A controlled trial of arthroscopic surgery for osteoarthritis of the knee, N Engl J Med 347:81, 2002. Scott WN, Clarke HD: Early knee arthritis: the role of arthroscopy, Orthopedics 26:943, 2003. Wai EK, Kreder HJ, Williams JI: Arthroscopic debridement of the knee for osteoarthritis in patients fifty years of age or older, J Bone Joint Surg 84(A):17, 2002. Wang C et al: Therapeutic effects of hyaluronic acid in osteoarthritis of the knee, J Bone Joint Surg 86A:538, 2004. Wegman A et al: Nonsteroidal antiinflammatory drugs or acetaminophen for osteoarthritis of the hip or knee? A systematic review of evidence and guidelines, J Rheumatol 31:344, 2004. AUTHOR: LONNIE R. MERCIER, M.D.

DISEASES AND DISORDERS

DIAGNOSIS

Section I

BASIC INFORMATION

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Osteomyelitis

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BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Osteomyelitis is an acute or chronic infection of the bone secondary to the hematogenous or contiguous source of infection or direct traumatic inoculation, which is usually bacterial.

• • • •

SYNONYMS

• • • •

Bone infection ICD-9CM CODES 730.1 Chronic osteomyelitis 730.2 Acute or subacute osteomyelitis

EPIDEMIOLOGY & DEMOGRAPHICS PREDOMINANT SEX: Male  female PREDOMINANT AGE: All ages

PHYSICAL FINDINGS HEMATOGENOUS OSTEOMYELITIS: • Localized inflammation: often secondary to trauma with accompanying hematoma or cellulitis • Fever • Lethargy • Irritability • Pain in involved bone VERTEBRAL OSTEOMYELITIS: Usually hematogenous. • Fever: 50% • Localized pain/tenderness • Neurologic defects: motor/sensory CONTIGUOUS OSTEOMYELITIS: DIRECT INOCULATION. • Associated with trauma, fractures, surgical fixation, pressure ulcer • Chronic infection of skin/soft tissue • Fever, drainage from surgical site CHRONIC OSTEOMYELITIS: • Bone pain • Sinus tract drainage, nonhealing ulcer • Chronic low-grade fever • Chronic localized pain

Bone infarction Charcot’s joint Gout Fracture

WORKUP ESR, C-reactive protein Blood culturing Bone culture Pathologic evaluation of bone biopsy for acute/chronic changes consistent with necrosis or acute inflammation

IMAGING STUDIES • • • • • •

Bone x-ray examination Bone scan (Fig. 1-26) Gallium scan Indium scan MRI (most accurate imaging study) Doppler studies: useful in patients with suspected peripheral vascular disease to determine vascular adequacy

TREATMENT Surgical debridement in biopsypositive cases will guide direction for antibiotic therapy. This will vary with type of osteomyelitis. Duration of therapy is usually 6 wk for acute osteomyelitis; chronic osteomyelitis may need a longer course of medication.

• S. aureus: cefazolin IV, nafcillin IV, vancomycin IV (in patient allergic to penicillin) • S. aureus (methicillin resistant): vancomycin IV • Streptococcus spp.: cefazolin or ceftriaxone • P. aeruginosa: piperacillin plus aminoglycoside or ceftazidime plus aminoglycoside • Enterobacteriaceae: ceftriaxone or fluoroquinolone • Hyperbaric oxygen therapy: may be useful in treatment of chronic osteomyelitis, especially with associated wound healing • Surgical debridement of all devitalized bone and tissue • Immobilization of affected bone (plaster, traction) if bone is unstable • Bone grafts using a vascularized or open graft may be necessary if the remaining bone is inadequate

SUGGESTED READINGS Boutin RD et al: Update on imaging of orthopedic infections, Orthop Clin North Am 29:41, 1998. Carek PJ et al: Diagnosis and management of osteomyelitis, Am Fam Physician 63:2413, 2001. AUTHORS: GLENN G. FORT, M.D., and DENNIS J. MIKOLICH, M.D.

ETIOLOGY • • • • • • • • • •

Staphylococcus aureus S. aureus (methicillin-resistant) Pseudomonas aeruginosa Enterobacteriaceae Streptococcus pyogenes Enterococcus Mycobacteria Fungi Coagulase-negative staphylococci Salmonella (in sickle cell disease)

FIGURE 1-26 Osteomyelitis. Intense accumulation of Tc-99m WBC in proximal phalanx of fifth digit of left foot at 4 hours after injection. (From Specht N [ed]: Practical guide to diagnostic imaging, St Louis, 1998, Mosby.)

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Osteoporosis BASIC INFORMATION

SYNONYMS None, but osteopenia describes a milder phase of this disease ICD-9CM CODES 733.0 Osteoporosis

EPIDEMIOLOGY & DEMOGRAPHICS • 44 million adults in the U.S. have abnormally low bone mass (osteopenia or osteoporosis). • 40% of all women and 25% of all men will experience a fragility (or low-impact) fracture in their lifetime. • 80% of all osteoporotic persons are women. • Osteoporosis (and osteopenia) account for 800,000 vertebral compression fractures, 300,000 hip fractures, and 250,000 wrist fractures, annually. • The consequences of those fractures are significant for the patients:  The mortality for hip fracture is 24% in the first 12 months; of those who survive, 50% fail to regain full ambulatory capability and 25% need long-term institutional care.  Vertebral fractures can be asymptomatic but they can also be associated with back pain (twofold increased risk of back pain associated with vertebral fractures), reduced activity or bed rest, 70,000 annual U.S. hospital admissions, and increased mortality. • Osteoporotic fractures are responsible for 4.1 million hospital days, 44.6 million nursing home days, and 3.4 million outpatient visits and an economic impact of $20 billion annually.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Often silent with no signs or symptoms. The prevalence of osteoporosis is 22% in white women ages 60 to 69, 38% in those ages 70 to 79, and 70% in those older than 80. This means that osteoporosis should be presumed to exist in all elderly women until proven otherwise. • The most common sign of osteoporosis is height loss. All elderly women (and probably men also) should be measured yearly. Most people know their adult height (or what it used to be). • Other signs include thoracic kyphosis (dowager’s hump), back pain associated with vertebral compression fractures (osteoporosis without resulting fractures does not cause back pain), symptoms/signs associated with the causes of secondary osteoporosis.

ETIOLOGY PRIMARY OSTEOPOROSIS Multifactorial resulting from a combination of factors including nutrition, peak bone mass, genetics, level of physical activity, age of menopause (spontaneous vs. surgical), and estrogen status. Postmenopausal osteoporosis is by far the most common form of primary osteoporosis. Increased bone resorption results in net bone loss, most rapidly during the first 5 years following menopause. In men, primary osteoporosis is also related to decreasing levels of sex hormones.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Differential diagnosis of low bone density: none Differential diagnosis of fractures: • Traumatic fracture resulting from a force that exceeds the strength of normal bone • Pathologic fractures involving a bone whose strength is weakened by a disease other than osteoporosis including:  Primary bone cancer  Metastatic bone cancer  Benign bone tumor  Paget’s disease  Osteomalacia  Osteopetrosis and other acquired causes of brittle bone • Osteoporotic (or fragility or low impact) fractures

WORKUP Diagnosis of osteoporosis is made by densitometry. It is indicated in: • All women 65 and over • Postmenopausal women under age 65 with osteoporosis risk factors • All adults with fragility fractures • Anyone expected to be treated with glucocorticoids drugs for longer than 3 months (cumulative)

DISEASES AND DISORDERS

Osteoporosis is a systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue resulting in reduced bone strength and increased risk of fracture. Other definitions: • Bone strength: bone density plus bone quality (bone mineral density accounts for 70% of bone strength) • Bone density: grams of mineral per volume • Bone mineral density: grams of mineral per area • Bone quality: factors that influence bone strength, including microarchitecture, turnover, damage accumulation, and mineralization • Microarchitecture: describes number of trabeculae, trabecular thickness, trabecular spacing, etc. Note: macroarchitecture also affects bone strength but is not related to osteoporosis.

SECONDARY OSTEOPOROSIS • Endocrine/metabolic  Vitamin D deficiency  Primary hyperparathyroidism  Thyrotoxicosis  Hypercortisolism (Cushing’s syndrome)  (Male) hypogonadism  Type I diabetes mellitus  Anorexia nervosa  Hyperprolactinemia  Porphyria  Hypophosphatasia • Gastrointestinal  Malabsorption syndromes  Chronic liver disease  Inflammatory bowel disease • Malignancies  Multiple myeloma  Lymphoma  Paraneoplastic syndromes  Other malignancies • Rheumatoid arthritis and variants • COPD • Osteogenesis imperfecta • Marfan’s syndrome • Homocystinuria • Mastocytosis • Drugs  Glucocorticoids  Thyroid hormones  Antiepileptic drug (e.g., phenytoin and phenobarbital)  Loop diuretics  GNRH antagonists

Section I

DEFINITION

RISK FACTORS FOR PRIMARY OSTEOPOROSIS AND FRAGILITY FRACTURES • Personal history of a prior fragility fracture is the strongest predictor of a subsequent fracture regardless of bone density • Family history of fracture • Cigarette smoking • Low body mass index/poor nutrition • Female gender • Early menopause (natural or surgical) • Age • Alcoholism • Low calcium intake • Sedentary life style • General fragility • Race/ethnicity (Whites and Asians at higher risk than African Americans and Hispanics.) • “Excessive” caffeine intake • History of falls • Unsteady gait • Risk factors for falls and unsteady gait such as impaired vision, vertigo, orthostatic hypotension, arthritis, neurologic disorders, dementia medications

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ture and should lead to consideration of active therapy to prevent further bone loss. A Z score of more than 1 to 2 standard deviations below the age-matched mean value should prompt a thorough evaluation for secondary causes of bone loss. The workup for secondary causes of osteoporosis is presented in Table 1-10.

RESISTIVE EXERCISES • Walking • Rowing machine • Weight lifting • Evidence suggests that the strongest predictor of compliance is enjoyability, which therefore should always be considered in providing recommendations. FALL PREVENTION • Assess gait and fall risk • Consider referral for gait training, lower extremity muscle strengthening, and balance training • Home safety evaluation • Assistive devices as appropriate • Hip protectors

TREATMENT See Table 1-11. The goal of treatment is fracture prevention. The strongest predictors of fracture are previous fracture, falls, low BMD, and advancing age (see Fig. 1-28).

Lumbar Spine Reference Database

T score

Z  2

2 BMD

• Selectively in adults with known diseases associated with secondary osteoporosis • Men 70 and older (controversial) TECHNOLOGIES FOR MEASURING BONE MINERAL DENSITY (BMD) • Quantitative ultrasound (QUS)  Advantages: 1. Low cost 2. Safe (no radiation)  Disadvantages 1. Only peripheral sites (finger, wrist, heel) 2. Imprecise • Dual-energy x-ray absorptiometry (DXA)  Advantages 1. The “gold standard” 2. Epidemiologic studies have correlated and standardized BMD data obtained by DXA with fracture risk 3. Precise 4. Can measure peripheral and central sites (hip and spine)  Disadvantages 1. More costly than QUS 2. Radiation exposure 3. Variable results among different machines 4. Any dense structure between radiation source and film (e.g., osteophytes, vascular calcifications) will give a false high reading, because density is measured from a two-dimensional image • Quantitative computed tomography (QCT)  Advantages 1. Central or peripheral site 2. Precise 3. Measures the intended targeted bone only as a three-dimensional structure  Disadvantages 1. Most expensive technology 2 Radiation exposure 3. Not yet fully standardized INTERPRETATION • All BMD measurement results are expressed in T scores and Z scores regardless of what technology is used. • World Health Organization Criteria for the Diagnosis of Osteoporosis  Normal: T-score 1  Osteopenia: T-score 1 to 2.5  Osteoporosis: T-score 2.5  Severe osteoporosis: T-score 2.5 plus fragility fracture(s) T SCORES AND Z SCORES See Fig. 1-27. DXA values are reported by comparison to age and gender reference groups with T scores (standard deviations or percentage above or below values for young normals) and Z scores (standard deviations or percentage above or below agematched values). A T score more than 2 standard deviations below young normals indicates an increased risk of frac-

Z0

0

Z  2

2 Z0

Z  2 4

Z  2

10

20

30

40

50

60

70

80

Age FIGURE 1-27 Natural history of lumbar spine bone mineral density, lumbar spine in women age 10 to 85. The band is a mean 2 standard deviation representation of bone density. The X-axis represents age; the Y-axis represents bone density in T scores. The middle of the ribbon represents a Z score of 0 (i.e., the statistical average woman’s bone density as she ages).

20 Fracture probability (%)

146

Age (yr) 80 70

10 60

50 0

3

2

1 T-score (SD)

0

1

FIGURE 1-28 Ten-year probability (%) of hip fracture in women according to age and T score for bone mineral density at the femoral neck. (Reproduced from Osteoporosis International, 13: 527-536, 2002 with permission. All rights reserved.)

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Osteoporosis Calcium preparations 1. Calcium carbonate 2. Calcium citrate 3. Calcium gluconate • Vitamin D: 800 IU per day from diet, sun exposure, or supplements. Many calcium supplements also contain vitamin D

PHARMACOLOGIC THERAPY • Indication for treatment (according to the National Osteoporosis Foundation)  T score 2.0 (by central DXA)  T score 1.5 (by central DXA) plus at least one additional fracture risk factor

Workup for the Secondary Cause of Osteoporosis (Low Z Score)

Differential Diagnosis

Screening

Positive Screen

Confirmatory Test

• Hyperparathyroidism • Low vitamin D state* • Hypercortisolism

Ca

, Phos Ca

, Phos FBS, K

↑ Ca

and/or ↓ Phos Lowish Ca

and ↓ Phos ↑ FBS, ↓ K

• Hyperthyroidism • Hypogonadism

TSH Testosterone, FSH, LH in men

↓ TSH ↓ testo, ↑ FSH, ↑ LH ↑ FSH, ↑ LH

Parathormone (PTH) level 25-OH-vit D level overnight dexamethasone suppression test TT4, TT3, T3 RU

CBC, ESR, Ca

, creat CBC ? Imaging CBC N/A

Anemia, ↑ ESR, ↑ Ca

or ↑ crest Anemia, lymphadenopathy ↑ WBC

SPEP, UEP More imaging Bone marrow aspirate

SGPT, Alk Phos, PT Creat Steroid use (for longer than 3 months)

Abn LFTs ↑ Creat

Variable Variable

CBC, PT, Carotene

Anemia, ↑ PT, ↓ carotene

D-xylose

Endocrine/Metabolism

Neoplasia • • • •

Multiple myeloma Lymphoma Leukemia Metastatic cancer

Chronic Illness • Hepatic failure • Renal failure • Rheumatoid arthritis Gastrointestinal • Malabsorption

absorption anemia evaluation

Drugs • • • •

Glucocorticoids Thyroid hormone Loop diuretics Dilantin

Medication history

*In high-risk populations (e.g., nursing home, northern latitude residence) a screening 25-OH-vit D level is recommended.

TABLE 1-11

Interventions for the Prevention and Treatment of Postmenopausal Osteoporosis Therapy

Dosage

Calcium Vitamin D Exercise (weight bearing)

1500 mg/d 800 IU/d 30 min, 3 days/wk

Risedronate Alendronate

5 mg/d or 35 mg/wk (prevention and treatment) 5 mg/d or 35 mg/wk (prevention) 10 mg/d or 70 mg/wk (treatment) 60 mg/d (prevention and treatment) 200 IU/d intranasally 50-100 IU/d SC or IM (treatment) 0.625 mg/d conjugated estrogens in women with bothersome menopausal symptoms 20 mcg/d SC (treatment)

Nonpharmacologic Measures

Pharmacologic Therapies

Raloxifene Salmon calcitonin HRT/ERT Teriparatide

DISEASES AND DISORDERS

TABLE 1-10



Section I

SUPPLEMENTS • Calcium: provide 1500 mg per day from diet or supplements.  Examples of dietary sources include: 1. Milk  300 mg per cup 2. Yogurt  350 mg per cup 3. Cheese  200 mg per oz 4. Broccoli  100 mg per cup

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Osteoporosis 

Any prior history of fragility vertebral or hip fracture: such patients should be treated even if a DXA is not available. • Preventive treatment for osteoporosis and treatment of osteopenia are controversial. • Antiresorptive (i.e., antiosteoclastic) treatments  Estrogen: effective at the dose of conjugated estrogens 0.625 mg daily in preventing hip and vertebral fractures; however, the overall risk outweighs the benefit. This treatment is no longer recommended for osteoporosis either as single therapy or combined with progesterone.  Selective estrogen receptor modulators (SERM): two drugs, raloxifen 60 mg daily and tamoxifen 20 mg daily, are available in the U.S.; however, only raloxifen is approved for the treatment (and prevention) of osteoporosis. Evidence exists that raloxifen is effective in reducing the risk of vertebral fractures. It is not known if it can reduce the risk of hip fracture. Both SERMs reduce the risk of breast cancer. Side effects are hot flashes, leg cramps, and increased risk of deep vein thrombosis and of endometrial cancer.  Calcitonin nasal spray 200 IU per day reduces the risk of vertebral fractures. It is not known if it can reduce the risk of hip fracture. The drug is well tolerated, but probably less effective than other agents.  Bisphosphonates: several bisphosphonates are available, but only three are widely used from the treatment or prevention of osteoporosis. They are alendronate (70 mg po weekly or 10 mg po daily), risedronate (35 mg po weekly or 5 mg po daily), and ibandronate (150 mg PO monthly). All three drugs are efficacious in reducing the risk of vertebral and hip fracture. The principal side effect for these drugs is potential esophageal ulceration and diarrhea. Alendronate may be marginally more potent at increasing bone density and risedronate may have better gastrointestinal tolerability, but both drugs are probably interchangeable. They are considered the first line of pharmacologic treatment for osteoporosis. • Bone-building treatments (i.e., osteoblastic stimulant): recombinant human parathyroid hormone (rh-PTH or teri-

paratide) is a potent stimulant of osteoblastic cells when administered as pulse therapy. (Continuous exposure to parathormone stimulates osteoclasts more than osteoblasts and results in a net bone loss.) Teriparatide is administered at the dose of 20 mcg injected SC daily and results in a reduction of both vertebral and nonvertebral fractures (not specifically hip as is the case for alendronate and risedronate). The main concern of teriparatide is its potential to cause osteosarcoma at high doses in rats. It is also very expensive. MONITORING TREATMENT • No evidence exists currently to demonstrate effectiveness of a monitoring strategy. Most clinicians recommend a central DXA at baseline, at 2 years, and at 4 years into treatment (the technology is not precise enough to recommend shorter intervals). Treatment success can be defined by an improvement or no change in BMD over time and no fracture. However, a decline in BMD over time does not necessarily indicate failure: treatment trials show efficacy in fracture reduction compared with placebo even in subjects with declining BMD. Nonetheless, many clinicians consider declining BMD or fracture to be indicative of treatment failure and need to change treatment. Evidence to support combined treatment is not convincing. • Markers of bone turnover are used by some clinicians to monitor response to treatment. The most common markers of bone resorption include acid phosphatase, hydroxyproline, and Ntelopeptide (a metabolite of collagen cross-links). The most common markers of bone formation are alkaline phosphatase and osteocalcin. Change in those markers only takes 3 months to observe, but such changes correlate only poorly with fracture risk. OSTEOPOROSIS IN MEN Twenty percent of all osteoporotic persons in the U.S. are men. Secondary causes (e.g., hypogonadism or vitamin D deficiency) are more likely than in women who typically have primary osteoporosis. Bisphosphonates, calcitonin, and teriparatide are used to treat osteoporotic men; however, only alendronate and teriparatide are approved. DURATION OF TREATMENT Most treatment trials were not conducted for periods longer than 5 years (alendronate has 10-yr data from trial exten-

sion open label studies). Some clinicians hesitate to treat for longer than 5 years (for lack of any evidence base), whereas others treat indefinitely because BMD declines after treatment withdrawal.

SUGGESTED READINGS Bone HG et al: Ten years’ experience with alendronate for osteoporosis in postmenopausal women, N Engl J Med 350:1189-1199, 2004. Cauley JA et al: Effects of estrogen plus progestin on risk of fracture and bone mineral density: the Women’s Health Initiative randomized trial, JAMA 290:1729-1738, 2003. Elliott ME et al: Fracture risks of women in long-term care: high prevalence of calcaneal osteoporosis and hypovitaminosis D, Pharmacotherapy 23:702-710, 2003. Greenspan SL et al: Alendronate improves bone mineral density in elderly women with osteoporosis residing in long-term care facilities: a randomized, double-blind, placebo-controlled trial, Ann Intern Med 136:742-746, 2002. Jolly EE et al: Prevention of osteoporosis and uterine effects in postmenopausal women taking raloxifene for 5 years, Menopause 10:337-344, 2003. Keller MI: Treating osteoporosis in postmenopausal women: a case approach, Cleve J Med 71:829, 2004. Kiebzak GM, Miller PD: Determinants of bone strength, J Bone Miner Res 18:383-384, 2003. Lindsay R et al: Risk of new vertebral fracture in the year following a fracture, JAMA 285:320-323, 2001. Marshall D, Johnell O, Wedel H: Meta-analysis of how well measures of bone mineral density predict occurrence of osteoporotic fractures, BMJ 312:1254-1259, 1996. National Osteoporosis Foundation: Physician’s Guide to Prevention and Treatment of Osteoporosis, Washington, DC, 2003, National Osteoporosis Foundation. Neer RM et al: Effect of parathyroid hormone (1-34) on fractures and bone mineral density in postmenopausal women with osteoporosis, N Engl J Med 344:1434-1441, 2001. Roux C et al: Efficacy of risedronate on clinical vertebral fractures within six months, Curr Med Res Opin 20:433-439, 2004. Siris ES et al: Identification and fracture outcomes of undiagnosed low bone mineral density in postmenopausal women: results from the National Osteoporosis Risk Assessment, JAMA 286:2815-2822, 2001. Sorensen OH et al: Long-term efficacy of risedronate: a 5-year placebo-controlled clinical experience, Bone 32:120-126, 2003. Zimmerman SI et al: The prevalence of osteoporosis in nursing home residents, Osteoporos Int 9:151-157, 1999. AUTHOR: TOM J. WACHTEL, M.D.

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Ovarian Cancer BASIC INFORMATION

DIAGNOSIS

SYNONYMS

DIFFERENTIAL DIAGNOSIS

Epithelial ovarian cancer Germ cell tumor Sex cord stromal tumor Ovarian tumor of low malignant potential

• • • • • • •

ICD-9CM CODES 183.0 Malignant neoplasm of ovary

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: 12.9 to 15.1 cases/100,000 persons; approximately 25,000 new cases annually PREDOMINANCE: Median age of 61 yr, peaks at age 75 to 79 yr (54/100,000) GENETICS: Familial susceptibility has been shown with the BRCA1 gene located on 17q12 to 21. This correlates with breast-ovarian cancer syndrome. RISK FACTORS: Low parity, delayed childbearing, use of talc on the perineum, high-fat diet, fertility drugs (possibly), Lynch II syndrome (nonpolyposis colon cancer, endometrial cancer, breast cancer, and ovarian cancer clusters in first- and second-degree relatives), breast-ovarian familial cancer syndrome, site-specific familial ovarian cancer (NOTE: Use of oral contraceptives appears to have a protective effect.)

PHYSICAL FINDINGS & CLINICAL PRESENTATION • 60% present with advanced disease • Abdominal fullness, early satiety, dyspepsia • Pelvic pain, back pain, constipation • Pelvic or abdominal mass • Lymphadenopathy (inguinal) • Sister Mary Joseph nodule (umbilical mass)

ETIOLOGY • Can be inherited as site-specific familial ovarian cancer (two or more firstdegree relatives have ovarian cancer)

Primary peritoneal cancer Benign ovarian tumor Functional ovarian cyst Ovarian torsion Pelvic kidney Pedunculated uterine fibroid Primary cancer from breast, GI tract, or other pelvic organ metastasized to the ovary

WORKUP • Definitive diagnosis made at laparotomy • Careful physical and history including family history • Exclusion of nongynecologic etiologies

LABORATORY TESTS • • • •

CBC Chemistry profile CA-125 or lysophosphatidic acid level Consider: hCG, Inhibin, AFP, neuronspecific enolase (NSE), and LDH in patients at risk for germ cell tumors • Osteopontin-Potential new biomarker for ovarian cancer

IMAGING STUDIES • Ultrasound (has not been shown to be effective as a screening mechanism but is useful in the evaluation of a pelvic mass) • Chest x-ray examination • Mammogram • CT scan to help evaluate extent of disease • Other studies (BE, MRI, IVP, etc.) as clinically indicated

TREATMENT NONPHARMACOLOGIC THERAPY Virtually all cases of ovarian cancer involve surgical exploration. This includes: • Abdominal cytology • Total abdominal hysterectomy and bilateral salpingo-oophorectomy

ACUTE GENERAL Rx • Optimal cytoreduction is generally followed by chemotherapy (except in some early-stage disease). • Cisplatin-based combination chemotherapy is used for stage II or greater, 6 mo treatment. • Chemotherapy regimens continue to change as research continues. • Consider second-look surgery when chemotherapy is complete.

CHRONIC Rx • If CA-125 have recurrent disease • Physical and pelvic examinations every 3 mo for 2 yr, every 4 mo during third year, then every 6 mo • CA-125 every visit • Yearly Pap smear

DISPOSITION • Overall 5-yr survival rates remain low because of the preponderance of latestage disease: Stage I and II 80% to 100% Stage III 15% to 20% Stage IV 5%

REFERRAL • Studies have shown that optimal cytoreduction is most likely to occur in the hands of a gynecologic oncologist. • Have gynecologic/oncology backup available if suspicious of malignancy.

SUGGESTED READINGS Haber D: Prophylactic oophorectomy to reduce the risk of ovarian and breast cancer in carriers of BRCA mutations, N Engl J Med 346:1660, 2002. Kim JH et al: Osteopontin as a potential diagnostic biomarker for ovarian cancer, JAMA 287:1671, 2002. Modan B et al: Parity, oral contraceptives and the risk of ovarian cancer among carriers and noncarriers of a brca1 or brca2 mutation, N Engl J Med 345:235, 2001. Olson SH et al: Symptoms of ovarian cancer, Obstet Gynecol 98:212, 2001. AUTHOR: GIL FARKASH, M.D.

DISEASES AND DISORDERS

Ovarian tumors can be benign, requiring operative intervention but not recurring or metastasizing; malignant, recurring, metastasizing, and having decreased survival; or borderline, having a small risk of recurrence or metastases but generally having a good prognosis.

• Omentectomy • Diaphragm sampling • Selective lymphadenectomy (pelvis and paraaortic) • Primary cytoreduction with a goal of residual tumor diameter 2 cm • Bowel surgery, splenectomy if needed to obtain optimal ( 2 cm) cytoreduction

Section I

DEFINITION

• Breast-ovarian cancer syndrome (clusters of breast and ovarian cancer among first- and second-degree relatives) • Lynch syndrome • No family history and unknown etiology in the majority of ovarian cancer cases

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Paget’s Disease of the Bone BASIC INFORMATION DEFINITION Paget’s disease of the bone is a nonmetabolic disease of bone characterized by repeated episodes of osteolysis and excessive attempts at repair that results in a weakened bone of increased mass. Monostotic (solitary lesion) and polyostotic (numerous lesions) disease are both described.

SYNONYMS Osteitis deformans ICD-9CM CODES 731.0 Paget’s disease (osteitis deformans)

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Localized lesions in 3% of patients 50 yr PREVALENT SEX: Male:female ratio of 2:1

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Many lesions are asymptomatic. • Onset is variable. • Symptoms result mainly from the effects of complications: 1. Skeletal pain, especially hip and pelvis 2. Bowing of long bones, sometimes leading to pathologic fracture 3. Increased heat of extremity (resulting from increased vascularity) 4. Skull enlargement and spinal involvement caused by characteristic bone enlargement, which can produce neurologic complications (vision, hearing loss, radicular pain, and cord compression) 5. Thoracic kyphoscoliosis 6. Secondary osteoarthritis, especially of hip 7. Heart failure as a result of chest and spine deformity and blood shunting

• Normal serum calcium and phosphorus levels • Increased urinary excretion of pyridinoline cross-links, although test is expensive and not usually required in routine cases • Other: bone biopsy only in uncertain cases or if sarcomatous degeneration is suspected

IMAGING STUDIES • Appropriate radiographs reflect the characteristic radiolucency and opacity (Fig. 1-29). • Bone scanning usually reflects the activity and extent of the disease.

TREATMENT ACUTE GENERAL Rx • Counseling regarding home environment to prevent falls • Cane for balance and weight-bearing pain

PHARMACOLOGIC THERAPY • • • •

Calcitonin Bisphosphonates NSAIDs for pain relief General indications for treatment 1. All symptomatic patients 2. Asymptomatic patients with high level of metabolic activity or those at risk for deformity 3. Preoperative, if surgery involves pagetic site

DISPOSITION • Many monostotic lesions probably remain asymptomatic. • Progression of the disease is common. • Malignant degeneration occurs in 1% of patients and should be considered when there is a sudden increase in pain.

• Sarcomatous change carries a grave prognosis.

REFERRAL • For dental evaluation if there is involvement of the mandible or maxilla • For ENT evaluation if there is hearing loss • For ophthalmologic evaluation if there is impaired vision • For orthopedic consultation for assessment of pain in bone or joint

PEARLS & CONSIDERATIONS COMMENTS Surgical intervention is often required for neurologic complications or joint symptoms • Often associated with profuse blood loss • Elective cases: benefit from preoperative treatment to suppress bone activity and vascularity

SUGGESTED READINGS Crandall C: Risedronate: a clinical review, Arch Intern Med 161:353, 2001. Kotocvicz MA: Paget’s disease of bone: Diagnosis and indications for treatment, Aust Fam Physician 33(3):127, 2004. Langston AL, Ralston SH: Management of Paget’s disease of bone, Rheumatology 43(8):955, 2004. Lin JT, Lane JM: Bisphosphonates, J Am Acad Orthop Surg 11:1, 2003. Roodman GD: Studies in Paget’s disease and their relevance to oncology, Semin Oncol 28:15, 2001. Schneider D et al: Diagnosis and treatment of Paget’s disease of bone, Am Fam Physician 65:2069, 2002. AUTHOR: LONNIE R. MERCIER, M.D.

ETIOLOGY Unknown

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Fibrous dysplasia • Skeletal neoplasm (primary or metastatic) • Osteomyelitis • Hyperparathyroidism • Vertebral hemangioma

LABORATORY TESTS • Increased serum alkaline phosphatase (SAP)

FIGURE 1-29 Frontal radiograph of the pelvis shows marked prominence to the trabeculae in the right ilium, ischium, and pubic bones with small lytic areas identified compatible with the later stages of Paget’s disease. (From Specht N [ed]: Practical guide to diagnostic imaging, St Louis, 1998, Mosby.)

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Pancreatic Cancer (Exocrine) BASIC INFORMATION Pancreatic cancer is an adenocarcinoma derived from the epithelium of the pancreatic duct.

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: 1 case/10,000 persons/yr PEAK AGE: Seventh and eighth decades of life PREDOMINANT SEX: Male:female ratio of 2:1

PHYSICAL FINDINGS & CLINICAL PRESENTATION Presenting symptoms: • Jaundice • Abdominal pain • Weight loss • Anorexia/change in taste • Nausea • Uncommonly: depression, GI bleeding, acute pancreatitis, back pain Physical findings: • Icterus • Cachexia • Excoriations from scratching pruritic skin

ETIOLOGY Unknown, but several conditions have been associated with pancreatic cancer: • Smoking • Alcoholism • Gallstones • Diabetes mellitus • Chronic pancreatitis • Diet rich in animal fat • Occupational exposures: oil refining, paper manufacturing, chemical industry

% abnormal 80 55 15 15 60

IMAGING STUDIES Noninvasive imaging Abdominal ultrasonography Abdominal CT scan (Fig. 1-30) (without or with contrast [IV or oral]) Abdominal MRI scan Invasive imaging Endoscopic retrograde cholangiopancreatography (ERCP) CT scan or ultrasonographyguided needle aspiration cytology

% abnormal 60 90

90 90

Palliative therapeutic endoscopic retrograde cholangiopancreatography (ERCP) using stents • Chemotherapy The best combination chemotherapy using streptozotocin, mitomycin C, and 5-FU provides only a 19-wk median survival. • Radiation External beam radiation for palliation of pain. • Combined chemotherapy and radiation provides a median survival of 11 mo. • Celiac plexus block by an experienced anesthesiologist provides pain relief in 80% to 90% of cases.

DISPOSITION Adjunct chemotherapy has a significant survival benefit in patients with resected pancreatic cancer, whereas adjuvant chemotherapy has a deleterious effect on survival.

90-95

TREATMENT • Surgery Curative pancreatectomy (Whipple’s procedure) appropriate for only 10% to 20% of patients whose lesion is 5 cm, solitary, and without metastases. Surgical mortality is 5%. Adjunct chemotherapy may improve postoperative survival. Palliative surgery (for biliary decompression/diversion)

SUGGESTED READINGS Cello JP: Pancreatic cancer. In Feldman M, Scharschmidt BF, Sleisenger MH (eds): Gastrointestinal and Liver Disease, ed 6, Philadelphia, 1998, WB Saunders. Michaud DS et al: Physical activity, obesity, height, and the risk of pancreatic cancer, JAMA 286:821, 2001. Neoptolemos JP et al: A randomized trial of chemotherapy and radiochemotherapy after resection of pancreatic cancer, N Engl J Med 350:1200, 2004. Wong GY: Effect of neurolytic celiac plexus block on pain relief, quality of life, and survival in patients with unresectable pancreatic cancer, JAMA 291:1092, 2004. AUTHOR: TOM J. WACHTEL, M.D.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • •

Common duct cholelithiasis Cholangiocarcinoma Common duct stricture Sclerosing cholangitis Primary biliary cirrhosis Drug-induced cholestasis (e.g., phenothiazines) • Chronic hepatitis • Sarcoidosis • Other pancreatic tumors (islet cell tumor, cystadenocarcinoma, epidermoid carcinoma, sarcomas, lymphomas)

FIGURE 1-30 CT scan of a patient with adenocarcinoma of the body and tail of the pancreas. The tumor (arrow) is seen anterior and adjacent to the left kidney (K). At operation, the tumor was invading Gerota’s fascia. (From Sabiston D: Textbook of surgery, ed 15, Philadelphia, 1997, WB Saunders.)

DISEASES AND DISORDERS

ICD-9CM CODES 157.9 Pancreatic cancer 157.0 (head) 157.1 (body) 157.2 (tail) 157.3 (duct) 230.9 (in situ)

Routine laboratory tests Alkaline phosphatase Bilirubin Total protein Amylase Hematocrit

Section I

DEFINITION

WORKUP

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SYNONYMS

becomes smaller, and hypophonia— voice becomes softer. • Postural instability—tested by “pull test.” Ask patient to stand in place with back to examiner. Examiner pulls patient back by the shoulders, and proper response would be to take no steps back or very few steps back without falling. Retropulsion is a positive test as is falling straight back. This is not usually severe early on. If falls and postural reflexes are greatly impaired early on, then consider other disorders.

Paralysis agitans

ETIOLOGY

BASIC INFORMATION DEFINITION Idiopathic Parkinson’s disease (PD) is a progressive neurodegenerative disorder characterized clinically by rigidity, tremor, and bradykinesia. The major manifestations of the disease are due to loss of dopamine in the substantia nigra pars compacta. Its pathologic hallmark is the Lewy body, which is a cytoplasmic eosinophilic inclusion body.

ICD-9CM CODES 332.0 Idiopathic Parkinson’s disease, primary 332.1 Parkinson’s disease, secondary

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE • Affects over 1 million people in North America • In age group 70 yr, 700/100,000 are affected • Highest incidence in whites, lowest incidence in Asians and black Africans

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Tremor—typically a resting tremor with a frequency of 4-6 Hz that is often first noted in the hand as a pillrolling tremor (thumb and forefinger). Can also involve the leg and lip. Tremor improves with purposeful movement. Usually starts asymmetrically but can eventually involve the other hemibody. • Rigidity—increased muscle tone. This, too, is usually asymmetric in onset, involving the arm, leg, or both. It is resistance that persists throughout the range of passive movement of a joint. • Akinesia/Bradykinesia—slowness in initiating movement. • Mask facies—face seems expressionless, giving the appearance of depression. Decreased blink, often there is excess drooling. • Gait disturbance. In the elderly, falls (particularly backwards) may be the first sign of PD. • Stooped posture, decreased arm swing. • Difficulty initiating the first step; small shuffling steps that increase in speed (festinating gait) as if the patient is chasing his or her center of gravity (steps become progressively faster and shorter while the trunk inclines further forward). • Other complaints and findings early on include micrographia—handwriting

• Primary  Unknown  Most cases are sporadic, with age being the most common risk factor, although there is probably a combination of both environmental and genetic factors contributing to disease expression. There are rare familial forms. • Secondary (acquired) parkinsonism  Postinfectious parkinsonism—von Economo’s encephalitis  Parkinson’s pugilistica—after repeated head trauma  Iatrogenic—any of the neuroleptics and antipsychotics. The high potency D2-blocker neuroleptics are most likely to cause parkinsonism. Quetiapine is an atypical antipsychotic with a lower risk of causing parkinsonism. Clozaril does not cause parkinsonism.  Toxins (e.g., manganese, carbon monoxide)  Cerebrovascular disease (basal ganglia infarcts)

DIAGNOSIS A presumptive clinical diagnosis can be based on history and physical examination. The combination of asymmetric signs, resting tremor, and good response to levodopa best differentiates idiopathic Parkinson’s disease from other causes of parkinsonism (see Differential Diagnosis).

DIFFERENTIAL DIAGNOSIS • Multisystem atrophy—distinguishing features include autonomic dysfunction, including urinary incontinence, orthostatic hypotension, and erectile dysfunction, parkinsonism, cerebellar signs, and normal cognition. • Diffuse Lewy body disease—parkinsonism with concomitant dementia. Patients often have early hallucinations

and fluctuations in level of alertness and mental status. • Corticobasal degeneration—often begins asymmetrically with apraxia, cortical sensory loss in one limb, and sometimes alien limb phenomenon. • Progressive supranuclear palsy—tends to have axial rigidity greater than appendicular (limb) rigidity. These patients have early and severe postural instability. Hallmark is supranuclear gaze palsy that involves vertical gaze before horizontal. Progressive dementia is part of this syndrome. • Essential tremor—bilateral postural and action tremor.

WORKUP Identification of clinical signs and symptoms associated with Parkinson’s disease (see Physical Findings & Clinical Presentation) and elimination of conditions that may mimic it with a comprehensive history and physical examination

IMAGING STUDIES CT scan has almost no role in investigations. MRI of the head may sometimes distinguish between idiopathic Parkinson’s disease and other conditions that present with signs of parkinsonism (see Differential Diagnosis).

TREATMENT NONPHARMACOLOGIC THERAPY • Physical therapy, patient education and reassurance, treatment of associated conditions (e.g., depression) • Avoidance of drugs that can induce or worsen parkinsonism: neuroleptics (especially high potency), certain antiemetics (prochlorperazine, trimethobenzamide), metoclopramide, nonselective MAO inhibitors (may induce hypertensive crisis), reserpine, methyldopa

PHARMACOLOGIC THERAPY • Whether levodopa should always be the initial treatment is controversial, but is the prevailing practice in the U.S. • Pharmacotherapy should be initiated when required by symptoms; prior practice of waiting for limitation of ADLs is now outdated. • Motor complications that develop during the course of the disease may reflect the combination of disease progression and side effects of dopaminergic medications.

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•

•

• •

DISPOSITION Parkinson’s disease usually follows a slowly progressive course leading to disability over the course of several years. However, every patient will progress individually and patients should be reassured that this diagnosis does not always result in debilitation.

REFERRAL • Neurology consultation is recommended on initial diagnosis of Parkinson’s disease. • Participation in outpatient physical therapy, occupational, or speech program is recommended for patients with moderate to advanced disease. Falls are a common problem and require special attention.

PEARLS & CONSIDERATIONS • Asymmetry of symptoms at onset is very useful in distinguishing PD from other causes of parkinsonism. • Although resting tremor is a common presenting symptom, up to one fourth of patients with idiopathic PD do not have classic resting tremor. • Management of delirium and hallucinations is often difficult because most antipsychotic drugs have extrapyramidal side effects that worsen Parkinsonian symptoms. The atypical antipsychotic drugs of choice in PD are Clozaril (watch for agranulocytosis) and quetiapine. • Walkers, if prescribed, should always be wheeled (to reduce the risk of falling backwards when lifting a nonwheeled walker).

COMMENTS Additional patient information on Parkinson’s disease can be obtained from www.parkinson.org and from the National Parkinson Foundation, Inc., 1501 Ninth Avenue NW, Miami, FL 33136; phone: (800) 327-4545.

SUGGESTED READINGS Ahlskog, JE: Parkinson’s disease: medical and surgical treatment, Neurol Clin 19:3, 2001. Lang AE et al: Parkinson’s disease, N Engl J Med 339(15):1044, 1998. Lang AE et al: Parkinson’s disease, N Engl J Med 339(16):1130, 1998. Siderowf A: Parkinson’s disease: clinical features, epidemiology, and genetics, Neurol Clin 19:3, 2001. AUTHOR: CINDY ZADIKOFF, M.D.

DISEASES AND DISORDERS

•

once advocated as early, first line therapy because of proposed neuroprotective effects; however, those benefits are probably less robust than once thought. Usual dose is 5 mg bid with breakfast and lunch. It can be useful in treating the fatigue that is commonly associated with PD. Concurrent use of stimulants and sympathomimetics should be avoided. Amantadine (Symmetrel) is an antiviral agent that augments release and decreases reuptake of dopamine. It can be used alone early in the disease or in combination with levodopa; dosage is 100 mg tid (titrate q week from 100 mg qd). Must adjust for age and renal impairment. Most notable side effect, especially in elderly, is confusion. Anticholinergic agents are helpful in treating the tremor and drooling in patients with Parkinson’s disease and can be used alone or in combination with levodopa; potential side effects include constipation, urinary retention, memory impairment, and hallucinations. They should generally be avoided in the elderly. 1. Trihexyphenidyl (Artane): initial dose, 1 mg tid po 2. Benztropine (Cogentin): usual dose, 0.5 to 1 mg qd or bid Surgical options: 1. Pallidal (globus pallidus interna) and subthalamic deep brain stimulation are currently the surgical options of choice; thalamic DBS may be useful for refractory tremor. 2. Surgery is limited to patients with disabling, medically refractory problems, and patients must still have a good response to L-dopa to undergo surgery. DBS results in decreased dyskinesias, fluctuations, rigidity, and tremor. Associated depression should be identified and treated. Coexisting dementia may represent either diffuse Lewy body disease or a late complication of PD. An acetylcholinesterase inhibitor trial should be prescribed.

Section I

• Levodopa therapy 1. Cornerstone of symptomatic therapy—should be used with a peripheral dopa decarboxylase inhibitor (carbidopa) to minimize side effects (nausea, mood changes, postural hypotension). The combination of the two drugs is marketed under the trade name Sinemet. 2. Usual starting dose is 25/100 mg (carbidopa/levodopa) tid 1 hr before meals. 3. Controlled-release preparations (Sinemet CR [200 mg levodopa/50 mg carbidopa, or 100 mg levodopa/25 mg carbidopa]) are available. • Entacapone (Comtan) is a reversible inhibitor of catechol-o-methyl transferase and is used as an adjunct to levodopa/carbidopa. Side effects include orthostatic hypotension, diarrhea, hallucinations. Not to be used with any nonselective MAO inhibitor. Initial dose is 200 mg tid given with each dose of Sinemet. • Dopamine receptor agonists (ropinirole, pramipexole, pergolide, and bromocriptine) are not as potent as levodopa, but they are often used as initial treatment in younger patients to attempt to delay the onset of complications (dyskinesias, motor fluctuations) associated with levodopa therapy. These medications are more expensive than levodopa. In general they cause more side effects than levodopa. These include nausea, vomiting, light-headedness, peripheral edema, confusion, and somnolence. 1. Ropinirole (Requip): initial dose is 0.25 mg tid 2. Pramipexole (Mirapex): initial dose of 0.125 mg tid 3. Pergolide (Permax): initial dose, 0.05 mg for first 2 days increased by 0.1 mg every third day over next 12 days. There have been cases of restrictive valvulopathy, most commonly in tricuspid valve, associated with Permax use. All patients on this medication need a good clinical cardiac examination, and if any concern, an echo 4. Bromocriptine (Parlodel): initial dose, 1.25 mg qhs • Selegiline (Eldepryl), an inhibitor of MAO B, can be used early as initial therapy in those with mild disease or as adjunctive therapy. Selegiline was

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Peptic Ulcer Disease BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Peptic ulcer disease (PUD) is an ulceration in the stomach or duodenum resulting from an imbalance between mucosal protective factors and various mucosal damaging mechanisms (see “Etiology”).

• • • • • •

SYNONYMS PUD Duodenal ulcer (DU) Gastric ulcer (GU) ICD-9CM CODES 536.8 Peptic ulcer disease 531.3 Peptic ulcer, stomach, acute 531.7 Peptic ulcer, stomach, chronic 532.3 Peptic ulcer, duodenum, acute 532.7 Peptic ulcer, duodenum, chronic

EPIDEMIOLOGY & DEMOGRAPHICS • Incidence: 250,000 to 500,000 (200,000 to 400,000 DU; 50,000 to 100,000 GU) annually; duodenal ulcer:gastric ulcer ratio is 4:1. • Anatomic location: 90% of DUs occur in the first portion of the duodenum; GU occurs most frequently in the lesser curvature near the incisura angularis.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Physical examination is often unremarkable. • Patient may have epigastric tenderness, tachycardia, pallor, hypotension (from acute or chronic blood loss), nausea and vomiting (if pyloric channel is obstructed), boardlike abdomen and rebound tenderness (if perforated), and hematemesis or melena (with a bleeding ulcer).

ETIOLOGY Often multifactorial; the following are common mucosal damaging factors: • Helicobacter pylori infection • Medications (NSAIDs, including aspirin, and glucocorticoids) • Incompetent pylorus or LES • Bile acids • Impaired proximal duodenal bicarbonate secretion • Decreased blood flow to gastric mucosa • Acid secreted by parietal cells and pepsin secreted as pepsinogen by chief cells • Cigarette smoking • Alcohol

GERD Cholelithiasis syndrome Pancreatitis Gastritis Nonulcer dyspepsia Neoplasm (gastric carcinoma, lymphoma, pancreatic carcinoma) • Angina pectoris, MI, pericarditis • Dissecting aneurysm • Other: high small bowel obstruction, pneumonia, subphrenic abscess, early appendicitis

WORKUP • Comprehensive history and physical examination to exclude other diagnoses. Diagnostic modalities include endoscopy or UGI series. Endoscopy is invasive and more expensive; however, it is preferred for the following reasons: 1. Highest accuracy (approximately 90% to 95%) 2. Useful to identify superficial or very small ulcerations 3. Essential to diagnose gastric ulcers (1% to 4% of gastric ulcers diagnosed as benign by UGI series are eventually diagnosed as gastric carcinoma) 4. Additional advantages over UGI series include: • Biopsy of suspicious looking ulcers • Electrocautery of bleeding ulcers • Measurement of gastric pH in suspected gastrinoma (e.g., patient with multiple ulcers) • Diagnosis of esophagitis, gastritis, duodenitis • Endoscopic biopsy for H. pylori

LABORATORY TESTS • Routine laboratory evaluation is usually unremarkable. • Anemia may be present in patients with significant GI bleeding. • H. pylori testing via endoscopic biopsy, urea breath test, stool antigen test (H. pylori stool antigen), or specific antibody test is recommended: 1. Serologic testing for antibodies to H. pylori is easy and inexpensive; however, the presence of antibodies demonstrates previous but not necessarily current infection. Antibodies to H. pylori can remain elevated for months to years after infection has cleared; therefore antibody levels must be interpreted in light of patient’s symptoms and other test results (e.g., PUD seen on UGI series). 2. The urea breath test documents active infection. The patient ingests a

small amount of urea labeled with carbon 13 (13C) or carbon 14. If urease is present (produced by the organism), the urea is hydrolyzed and the patient exhales labeled carbon dioxide that is then collected and measured. This test is more expensive and not as readily available. Use of proton pump inhibitors within 2 wk of the urea breath test may interfere with test results. 3. Histologic evaluation of endoscopic biopsy samples is currently the gold standard for accurate diagnosis of H. pylori infection. 4. Stool antigen test is as accurate as the urea breath test for follow-up evaluation of patients treated for H. pylori. This test detects the presence of infection by measuring the fecal excretion of H. pylori antigens. A negative result on the stool antigen test 8 wk after completion of therapy identifies patients in whom eradication of H. pylori was successful. • Additional laboratory evaluation is indicated only in specific cases (e.g., amylase level in suspected pancreatitis, serum gastrin level in suspected Zollinger-Ellison [Z-E] syndrome).

IMAGING STUDIES Conventional UGI barium studies identify approximately 70% to 80% of PUD; accuracy can be increased to approximately 90% by using double contrast.

TREATMENT NONPHARMACOLOGIC THERAPY • Stop cigarette smoking; cigarette smoking increases the risk of PUD, decreases the healing rate, and increases the frequency of recurrence. • Avoid NSAIDs and alcohol. • Special diets have been proved unrelated to ulcer development and healing; however, avoid foods that cause symptoms.

ACUTE GENERAL Rx Eradication of H. pylori, when present, can be accomplished with various regimens: 1. Proton pump inhibitors (PPI) bid (e.g., omeprazole 20 mg bid or lansoprazole 30 mg bid) plus clarithromycin 500 mg bid and amoxicillin 1000 mg bid for 7 to 10 days 2. PPI bid plus amoxicillin 500 mg bid plus metronidazole 500 mg for 7 to 10 days 3. PPI bid plus clarithromycin 500 mg bid and metronidazole 500 mg bid for 7 days

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Maintenance therapy in duodenal ulcer patients is indicated in the following situations: • Persistent smokers • Recurrent ulcerations • Chronic treatment with NSAIDs, glucocorticoids

DISPOSITION • The recurrence rate for untreated PUD is approximately 60% (70% in smokers). Treatment decreases the recurrence rate by nearly 30%. • Patients with recurrent ulcers should be retreated for an additional 8 wk and then placed on maintenance therapy with H2RAs, PPIs, sucralfate, or antacids. • An ulcer is considered refractory to treatment if healing is not evident after 8 wk for duodenal ulcers and 12 wk for gastric ulcers. In these patients maximum acid inhibition (e.g., esomeprazole 40 mg bid) is preferred over continued therapy with standard antiulcer therapy. • Eradication of H. pylori (when present) is indicated in all patients. A negative stool antigen test for H. pylori 6 weeks after treatment accurately confirms cure of H. pylori infection with reasonable sensitivity in initially seropositive healthy subjects. • Screening for Zollinger-Ellison (Z-E) syndrome should also be considered in patients with multiple recurrent ulcers; in patients with Z-E, the serum gastrin level is 1000 pg/ml and the basal acid output is usually 15 mEq/hr.

• Surgery for refractory ulcers is now only rarely performed; it consists of highly selective vagotomy for duodenal ulcers or ulcer removal with antrectomy or hemigastrectomy without vagotomy for gastric ulcers.

REFERRAL • GI referral for patients requiring endoscopy • Surgical referral for patients with nonhealing ulcers despite appropriate medical therapy

PEARLS & CONSIDERATIONS COMMENTS • Patients with gastric ulcers should have repeat endoscopy after 4 to 6 wk of therapy to document healing and test exfoliative cytology for gastric carcinoma. • After endoscopic treatment of bleeding peptic ulcers, bleeding recurs in up to 20% of patients. PPI administration intravenously by continuous infusion substantially reduces the risk of recurrent bleeding.

SUGGESTED READINGS Graham DY et al: Ulcer prevention in longterm users of nonsteroidal anti-inflammatory drugs, Arch Intern Med 162:169, 2002. Lai KC et al: Lansoprazole for the prevention of recurrences of ulcer complications from long-term low-dose aspirin use, N Engl J Med 346:2033, 2002. Lara LF et al: One day quadruple therapy compared with 7-day triple therapy for helicobacter pylori infection, Arch Intern Med 163:2079, 2003. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

CHRONIC Rx

• Elderly or debilitated patients • Aggressive or complicated ulcer disease (e.g., perforation, hemorrhage) • Asymptomatic bleeders Misoprostol therapy (100 g qid with food, increased to 200 g qid if well tolerated) is useful for the prevention of NSAID-induced gastric ulcers in all patients on long-term NSAID therapy. Proton pump inhibitors are also effective at healing ulcers and maintaining remission in patients on long-term NSAIDs.

Section I

4. Recent trials indicate that a 1-day quadruple therapy may be as effective as a 7-day triple therapy regimen. The 1-day quadruple therapy regimen consists of two tablets of 262 mg bismuth subsalicylate qid, one 500 mg metronidazole tablet qid, 2 g of amoxicillin suspension qid, and two capsules of 30 mg of lansoprazole. 5. Bismuth compound qid plus tetracycline 500 mg qid and metronidazole 500 mg qid for 14 days 6. A 5-day treatment with three antibiotics (amoxicillin 1 g bid, clarithromycin 250 mg bid, and metronidazole 400 mg bid) plus either lansoprazole 30 mg bid or ranitidine 300 mg bid is an efficacious costsaving option for patients older than 55 yr with no prior history of PUD PUD patients testing negative for H. pylori should be treated with antisecretory agents: • Histamine-2 receptor antagonists (H2RAs): cimetidine, ranitidine, famotidine, and nizatidine are all effective; they are usually given in split dose or at nighttime. • Proton pump inhibitors (PPIs): can also induce rapid healing; they are usually given 30 min before meals. Antacids and sucralfate are also effective agents for the treatment and prevention of PUD.

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Peripheral Arterial Disease BASIC INFORMATION DEFINITION Peripheral arterial disease (PAD) usually refers to atherosclerotic obstruction of the arteries to the lower extremity. ICD-9CM CODES 443.9 Peripheral vascular disease

EPIDEMIOLOGY & DEMOGRAPHICS • Age-adjusted prevalence of PAD is approximately 12%. • PAD affects men and women equally. • An estimated 27 million people in Europe and North America (or 16% of the population 55 yr of age and older) have PAD. • African Americans and Hispanics with diabetes have a higher prevalence of PAD than whites. • PAD is a marker for systemic vascular disease. • Patients with newly diagnosed PAD are 6 times more likely to die within the next 10 yr when compared with patients without PAD. • Risk factors associated with PAD are similar to coronary artery disease, including tobacco, diabetes, hyperlipidemia, hypertension, and advanced age. • Smoking is the major determinant of disease progression. • Other potential risk factors include elevated levels of C-reactive protein, fibrinogen, homocysteine, apolipoprotein (a), and plasma viscocity. • An inverse relationship has been suggested between PAD and alcohol consumption.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Nearly 50% of the patients with PAD experience no symptoms, making PAD an underdiagnosed and undertreated condition • Approximately one third of patients with PAD present with intermittent claudication described as an aching or cramping leg pain brought on by exertion and relieved with rest that can progress with time; however, relying on the classic history of claudication alone will miss 85%-90% of patients with PAD • Pain at rest occurring commonly at night when the patient is supine • Diminished pulses • Bruits heard over the distal aorta, iliac, or femoral arteries • Rubor with prolonged capillary refill on dependency • Cool skin temperature

• Trophic changes of hair loss and muscle atrophy • Nonhealing ulcers, necrotic tissue, and gangrene possible

ETIOLOGY The primary cause of peripheral arterial disease is atherosclerosis: atherosclerotic lesions of the arteries to the lower extremities subsequently leading to stenosis of peripheral vessels and inability to supply oxygenated blood to working limb muscles.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Spinal stenosis • Degenerative joint disease of the lumbar spine and hips • Muscle cramps • Compartment syndrome

WORKUP • The initial workup in any patient suspected of having PAD includes measuring the ankle-brachial index (ABI). The ABI is calculated by dividing the highest ankle systolic pressure using either the dorsalis pedis or posterior tibial artery by the highest systolic pressure from either arm. • A diagnosis of PAD is based on the presence of limb symptoms or an ABI • The severity of PAD is based on the ABI at rest and during treadmill exercise (1 to 2 mph, 5 min, or symptomlimited) and is classified as follows: 1. Mild: ABI at rest 0.71 to 0.90 or ABI during exercise 0.50 2. Moderate: ABI at rest 0.41 to 0.70 or ABI during exercise 0.20 3. Severe: ABI at rest 0.40 or ABI during exercise 0.20

LABORATORY TESTS • • • • •

Lipid profile Blood glucose HgbA1c levels in diabetic patients Homocysteine Fibrinogen

IMAGING STUDIES • Duplex ultrasound can be used to locate the occluded areas and assess the patency of the distal arterial system or prior vein grafts. • Rest or exercise pulse volume recordings. Pulse volume recordings measure volume of limb flow per pulse in different segments of the limb (e.g., thigh, calf, ankle, metatarsal, and toes). It helps to localize the site of the stenosis since the contour of the pulse wave changes distal to the occlusion.

• MRA can be used as a noninvasive approach to visualize the aorta and peripheral lower extremity arteries. A major advantage of MRA is that it does not require contrast agents. • Angiography remains the gold standard for visualizing the arterial anatomy before revascularization.

TREATMENT NONPHARMACOLOGIC THERAPY • PAD patients with no prior history of a cardiac event are to be considered as a cardiovascular “equivalent” with risks of future cardiovascular events similar to patients with prior MIs • Diet counseling (e.g., salt restriction in hypertension, ADA calorie diets in diabetics) • Exercise training walking 30 to 60 min/day at about 2 mi/hr every day improves exercise capacity, walking distance, and quality of life • Aggressive management of risk factors for PAD including: 1. Tobacco counseling and smoke cessation programs are indispensable in decreasing the progression of disease as well as reducing the mortality rate from cardiovascular events in patients with PAD. 2. Management of hypertension. 3. Tight glycemic control (A1C <7%) in diabetic patients with PAD results in prevention of microvascular complications. 4. Control of dyslipidemia reduces severity of claudication symptoms.

ACUTE GENERAL THERAPY Most patients with PAD respond to conservative management mentioned previously. If this fails, medicines can be tried (see “Chronic Rx”). Surgical reconstitution has its specific indications reserved for patient with impending limb loss (see “Chronic Rx”).

CHRONIC Rx • Aspirin 81 mg to 325 mg daily is recommended for secondary disease prevention in patients with cardiovascular disease. • Clopidogrel 75 mg daily also provides protection from cardiovascular and cerebrovascular events associated with PAD. • Pentoxyphylline (Trental, Pentoxil) 400 mg tid may provide a small benefit in walking distance when compared with placebo.

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Risk factor modification with aggressive pharmacotherapy in the treatment of hyperlipidemia, diabetes, hypertension, and smoking is essential in the prevention of progression, limb ischemia, and cardiovascular events in patients with PAD.

SUGGESTED READINGS

Consultation with a vascular surgeon is recommended in patients with PAD and rest pain, functional disability from pain, ABI less than 0.50 at rest, any signs of limb ischemia, or gangrene.

American Diabetes Association: Peripheral arterial disease in people with diabetes, Diabetes Care 26:3333, 2003. Belch JJ et al: Critical issues in peripheral arterial disease detection and management, Arch Intern Med 163;884, 2003. Burns P, Gaugh S, Bradbury AW: Management of peripheral arterial disease in primary care, British Medical Journal 326:584, 2003. Hiatt WR: Medical treatment of peripheral arterial disease and claudication, N Engl J Med 344:21, 2001. Hirsch AT et al: Peripheral arterial disease detection, awareness, and treatment in primary care, JAMA 286:11, 2001. Lesho E et al: Management of peripheral arterial disease, Am Fam Physician 69:525, 2004. Mukheijee D, Yadav JS: Update on peripheral vascular diseases: from smoking cessation to stenting, Cleve Clinic J Med 68:8, 2001.

PEARLS & CONSIDERATIONS COMMENTS • Asymptomatic PAD, similar to symptomatic PAD, is associated with an increased risk of atherothrombotic events (e.g., MI and CVA). • Although the prevalence of PAD in Europe and North America is estimated at approximately 27 million people, PAD remains underdiagnosed and undertreated. When PAD limits a patient’s ability to walk and exercise, percutaneous revascularization can be considered. Data reveal excellent outcomes with angioplasty and stenting. Outcomes will likely be better as peripheral interventional technology and skills improve.

AUTHORS: PRANAV M. PATEL, M.D., and WEN-CHIH WU, M.D.

DISEASES AND DISORDERS

DISPOSITION

REFERRAL

Section I

• Cilostazol (Pletal) 100 mg bid has been shown to significantly increase the distance patients with claudication can walk when compared with placebo, but should not be given to patients with congestive heart failure and an ejection fraction 40. • Surgical reconstruction is indicated in patients with refractory rest pain, limb ischemia, nonhealing ulcers, or gangrene, and in a select group of patients with functional disability. Common surgical procedures: 1. Aortoiliofemoral reconstruction 2. Infrainguinal bypass (e.g., femoropopliteal, femorotibial) 3. Extraanatomic bypass (e.g., axillofemoral or femorofemoral bypass) • Angioplasty is used on short, discrete stenotic lesions in the iliac or femoropopliteal artery.

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Peripheral Nerve Dysfunction BASIC INFORMATION DEFINITION The term peripheral neuropathy refers to any disorder involving the peripheral nerves. It encompasses: • Polyneuropathy—a symmetric, usually length-dependent disorder of peripheral nerves; a distinction is often made between predominantly small-fiber (often painful) and large-fiber neuropathies. • Mononeuropathy—disorder of a single peripheral nerve (e.g., median or ulnar neuropathy). • Mononeuropathy multiplex—a multifocal disorder characterized by dysfunction of many individual peripheral nerves (when extensive, dysfunction may become confluent and resemble a generalized neuropathy). ICD-9CM CODES 356.9 Unspecified idiopathic peripheral neuropathy

PHYSICAL FINDINGS & CLINICAL PRESENTATION Polyneuropathy—symptoms usually begin distally in the feet and gradually spread proximally. • Sensory  Numbness, paresthesiae, neuropathic pain  Sensory ataxia (result of impairment of position sense)  Reduced or absent deep tendon reflexes • Motor  Weakness, muscle atrophy  Foot deformities (high arches, hammer toes)—especially with hereditary neuropathy • Autonomic  Postural hypotension Mononeuropathy—symptoms and signs depend on the peripheral nerve affected • Median nerve (carpal tunnel syndrome)—numbness, tingling in the thumb and adjacent two fingers; pain in the wrist and forearm; often worse at night and with activities like driving Mononeuropathy multiplex • Pain in the distribution of individual peripheral nerves • Multifocal motor and sensory deficits

ETIOLOGY HEREDITARY NEUROPATHIES • Charcot-Marie-Tooth syndrome 1. Most common familial motor and sensory neuropathy 2. Type I (most common) is demyelinating; type II is axonal 3. Foot deformities (high arches, hammer toes) are common ACQUIRED NEUROPATHIES • Demyelinating 1. Guillain-Barré syndrome—acute (progression over 4 wk) ascending flaccid paralysis with areflexia; may be accompanying sensory loss, pain, and autonomic dysfunction (see Guillain-Barré Syndrome in Section I) 2. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)— subacute to chronic (progression over 2 mo) sensorimotor polyneuropathy (may be associated with monoclonal gammopathy) • Axonal 1. Diabetes mellitus: frequently painful, usually affects sensory fibers more than motor 2. Nutritional deficiency—B12, folate 3. Toxins—alcohol, drugs (e.g., isoniazid, vincristine, cisplatin, colchicines, amiodarone, phenytoin, antiretrovirals, metronidazole, dapsone, nitrofurantoin, disulfiram), chemicals (lead, arsenic, mercury) 4. Thyroid disease (hypothyroidism) 5. Paraproteinemia—monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma, Waldenstrom’s macroglobulinemia, primary systemic amyloidosis 6. Collagen vascular disorders—SLE, rheumatoid arthritis, Sjögren’s syndrome, polyarteritis nodosa (may cause polyneuropathy and mononeuropathy multiplex) 7. Sarcoidosis: cranial nerve palsies (most common is facial nerve) 8. Paraneoplastic 9. Infections (leprosy, herpes zoster, TB, diphtheria, Lyme disease, HIV); may cause polyneuropathy or mononeuropathy 10. Uremia 11. Compression—typically causes mononeuropathy

DIAGNOSIS WORKUP • History should be directed toward inquiry about specific etiologies—systemic disease (e.g., diabetes mellitus, collagen disorder), alcohol intake, medication use, toxin exposure, dietary practices (e.g., veganism may cause B12 deficiency), risk factors for HIV infection, family history (of neuropathy or foot deformities). • Neurophysiology (nerve conduction studies and electromyography) to determine the pattern of involvement (e.g., polyneuropathy vs. mononeuropathy multiplex) as well as the physiology (axonal vs. demyelinating). • Lumbar puncture may be indicated when vasculitis or amyloid suspected. • Fig. 3-84 in Section III describes an approach to the patient with peripheral neuropathy.

LABORATORY TESTS Some or all of these tests may be appropriate, depending on clinical suspicion: • Fasting blood sugar and/or 2-hr glucose tolerance test • Serum B12, homocysteine, methylmalonic acid as well as folate • Renal function • Thyroid function tests • Serum protein and immunoelectrophoresis; urine protein electrophoresis • ANA, RF, cryoglobulins • HIV, RPR, hepatitis serologies, Lyme titers • Heavy metal screen; urinary porphyrins

IMAGING STUDIES Usually, imaging studies are not required. Certain clinical conditions may require the following: • Chest x-ray—when sarcoid or underlying lung cancer suspected • Skeletal survey (long bone x-rays) to identify plasmacytoma • MRI of lumbosacral (or cervical spine) if spinal cord pathology or polyradiculopathy suspected

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Peripheral Nerve Dysfunction TREATMENT

ACUTE GENERAL Rx • Treatment of underlying systemic disease (e.g., diabetes, uremia, multiple myeloma) • Nutritional replacement when appropriate (e.g., B12 injections)

Hughes RAC: Peripheral neuropathy, BMJ 324:466, 2002. Mendell JR, Sahenk Z: Clinical practice— painful sensory neuropathy, N Engl J Med 348:1243, 2003. Stevens JC et al: Symptoms of 100 patients with electromyographically verified carpal tunnel syndrome, Muscle Nerve 22:1448, 1999. AUTHOR: MICHAEL BENATAR, M.B.CH.B., D.PHIL.

DISEASES AND DISORDERS

if any identified. • Surgical referral for entrapment neuropathy. • Physical or occupational therapy as well as splinting (e.g., ankle-foot orthosis) as appropriate.

SUGGESTED READINGS Section I

NONPHARMACOLOGIC THERAPY • Stop offending agent (drugs or toxins)

• Symptomatic treatment of neuropathic pain—many options available:  Neurontin—start 100 mg tid (more slowly in the very elderly); gradually increase as needed and as tolerated, aiming initially for 600 mg tid.  Topamax—start 25 mg qd, then 25 mg bid, then 50 mg bid, increasing as tolerated and needed.  Lamictal—start 50 mg qd for 2 wk, then 50 mg bid for 2 wk; then increase by 50 mg per week as tolerated and needed (Stevens-Johnson rash may occur if dose escalates too quickly).  Narcotic analgesics—may be used, but the aforementioned agents should be tried first.

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Plantar Fasciitis BASIC INFORMATION DEFINITION Plantar fasciitis is a common, painful inflammation or degeneration of the plantar fascia, a tissue that extends from the calcaneus to the proximal phalanges of each toe.

SYNONYMS Painful heel syndrome Painful heel spur ICD-9CM CODES 728.71 Plantar fasciitis 726.73 Calcaneal spur

EPIDEMIOLOGY & DEMOGRAPHICS

ETIOLOGY

DISPOSITION

• Uncertain • Inflammation, microscopic tears, and/ or degeneration • The role of the calcaneal traction osteophyte (spur) is unclear • May be associated with tight heel cord

Disorder is usually self-limited, although full recovery may take 1 to 2 yr.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Other regional tendonitis Stress fracture Tarsal tunnel syndrome Tumor, infection

IMAGING STUDIES

PREVALENT SEX: Males  females Bilateral in 10% to 20% of cases

Traction osteophyte or minor soft tissue calcification may be present on plain radiography. Other studies are usually not required.

PHYSICAL FINDINGS & CLINICAL PRESENTATION

TREATMENT

• Pain is characteristically worse on arising and after periods of rest; “warming up” often lessens the pain • Local tenderness at site involvement, usually the medial tubercle of the calcaneus, sometimes in the midfascia • Pain sometimes elicited by passive dorsiflexion of toes and ankle, which stretches the plantar fascia • A tight heel cord may be present

• • • •

Sensible activity restriction Gentle stretching exercises NSAIDS Local steroid/lidocaine injections (Fig. 1-31) • Heel lift • Night brace, daytime cast brace

REFERRAL • If symptoms fail to respond to medical management • For surgical consideration (plantar fascia release, excision of osteophyte)

PEARLS & CONSIDERATIONS COMMENTS • Various cushions and heel cups are generally ineffective because stretching, not heel strikes, is probably cause of disorder. • Surgical intervention is rarely necessary. • Shock wave therapy is of unproven benefit.

SUGGESTED READINGS Aldridge J: Diagnosing heel pain in adults, Am Fam Physician 70:332, 2004. Bachbinder R et al: Ultrasound-guided extracorporeal shockwave therapy for plantar fasciitis, JAMA 288:1364, 2002. Bachbinder R: Plantar fasciitis, N Engl J Med 350:2159, 2004. DiGiovanni BF et al: Tissue specific plantar fascia stretching exercises enhances outcomes in patients with chronic heel pain, J Bone Joint Surg 85:1270, 2003. Haake M et al: Extra-corporeal shockwave therapy for plantar fasciitis: randomized controlled multicentre trial, Br Med J 327:75, 2003. Riddle DL et al: Risk factors for plantar fasciitis: a matched case-control study, J Bone Joint Surg 85:872, 2003. Rompe JD et al: Evaluation of low-energy extracorporeal shockwave application for chronic plantar fasciitis, J Bone Joint Surg 84(A):335, 2002. Young CC et al: Treatment of plantar fasciitis, Am Fam Physician 63:467, 2001. AUTHOR: LONNIE R. MERCIER, M.D.

FIGURE 1-31 Injection site for plantar fasciitis. Injection should be through the sole into the area of maximum tenderness. A 25- or 27-gauge needle should be used and the medication injected slowly as some pain may occur. The total volume should be no greater than 1.5 ml. (From Mercier L: Practical orthopedics, ed 5, St Louis, 2002, Mosby.)

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Pneumonia, Aspiration BASIC INFORMATION Aspiration pneumonia is a lung infection caused by bacterial organisms aspirated from nasopharyngeal space.

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • Few reliable data • 20% to 35% of all pneumonias • 5% to 15% of all community acquired pneumonias PREVALENCE (IN U.S.): Unreliable data PREDOMINANT SEX: Equal PREDOMINANT AGE: Elderly PEAK INCIDENCE: Elderly patients in hospitals or nursing homes

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Shortness of breath, tachypnea, cough, sputum, fever after vomiting, or difficulty swallowing • Rales, rhonchi, often diffusely throughout lung

ETIOLOGY Complex interaction of etiologies, ranging from chemical (often acid) pneumonitis following aspiration of sterile gastric contents (generally not requiring antibiotic treatment) to bacterial aspiration COMMUNITY-ACQUIRED ASPIRATION PNEUMONIA: • Generally results from predominantly anaerobic mouth bacteria (anaerobic and microaerophilic streptococci, fusobacteria, gram-positive anaerobic non–spore-forming rods), Bacteroides species (melaninogenicus, intermedius, oralis, ureolyticus), Haemophilus influenzae, and Streptococcus pneumoniae • Rarely caused by Bacteroides fragilis (of uncertain validity in published studies) or Eikenella corrodens • High-risk groups: elderly, alcoholics, IV drug users, patients who are obtunded, those with esophageal disorders, seizures, poor dentition, stroke victims, or recent dental manipulations HOSPITAL-ACQUIRED ASPIRATION PNEUMONIA: • Often occurs among elderly patients and others with diminished gag reflex; those with nasogastric tubes, intestinal obstruction, or ventilator support; and especially those exposed to contaminated nebulizers or unsterile suctioning • High-risk groups: seriously ill hospitalized patients (especially patients with coma, acidosis, alcoholism, uremia, di-

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Other necrotizing or cavitary pneumonias (especially tuberculosis, gramnegative pneumonias) • See “Pulmonary Tuberculosis”

WORKUP • • • •

Chest x-ray examination CBC, blood cultures Sputum Gram stain and culture Consideration of tracheal aspirate

LABORATORY TESTS • CBC: leukocytosis often present • Sputum Gram stain 1. Often useful when carefully prepared immediately after obtaining suctioned or expectorated specimen, examined by experienced observer. 2. Only specimens with multiple WBCs and rare or absent epithelial cells should be examined. 3. Unlike nonaspiration pneumonias (e.g., pneumococcal), multiple organisms may be present. 4. Long, slender rods suggest anaerobes. 5. Sputum from pneumonia caused by acid aspiration may be devoid of organisms. 6. Cultures should be interpreted in light of morphology of visualized organisms.

IMAGING STUDIES • Chest x-ray examination often reveals bilateral, diffuse, patchy infiltrates, in posterior segment upper lobes or at the bases.

TREATMENT NONPHARMACOLOGIC THERAPY • Airway management to prevent repeated aspiration—swallowing evaluation (may include a modified barium swallow test) • Ventilatory support if necessary • NPO and G-tube feeding do not effectively prevent aspiration

ACUTE GENERAL Rx Acute aspiration of acidic gastric contents without bacteria may not require antibiotic therapy; consult infectious diseases or pulmonary expert. FOR COMMUNITY-ACQUIRED ANAEROBIC ASPIRATION PNEUMONIA: • Levofloxacin 500 mg qd or ceftriaxone 1 to 2 g/day NURSING HOME ASPIRATIONS: • Levofloxacin 500 mg qd or piperacillin-tazobactam 3.375 g q6h or ceftazidime 2 g q8h HOSPITAL-ACQUIRED ASPIRATION PNEUMONIA: • Piperacillin-tazobactam 3.375 g IV q6h, or clindamycin 450-900 mg IV q8h, or cefoxitin 2 g IV q8h • Knowledge of resident flora in the microenvironment of the aspiration within the hospital is crucial to intelligent antibiotic selection; consult infection control nurses or hospital epidemiologist. • Confirmed Pseudomonas pneumonia should be treated with antipseudomonal -lactam agent plus an aminoglycoside until antimicrobial sensitivities confirm that less toxic agents may replace aminoglycoside. • Do not use metronidazole alone for anaerobes.

DISPOSITION Repeat chest x-ray examination in 6 to 8 wk.

REFERRAL For consultation with infectious disease and/or pulmonary experts for patients with respiratory distress, hypoxia, ventilatory support, pneumonia in more than one lobe, necrosis or cavitation on x-ray examination, or not responding to antibiotic therapy within 2 to 3 days

SUGGESTED READING Marik PE: Aspiration pneumonitis and aspiration pneumonia, N Engl J Med 344:665, 2001. AUTHOR: BETH J. WUTZ, M.D.

DISEASES AND DISORDERS

ICD-9CM CODES 507.0 Aspiration pneumonia

• Aspiration pneumonias of several days’ or longer duration may reveal necrosis (especially community-acquired anaerobic pneumonias) and even cavitation with air-fluid levels, indicating lung abscess.

Section I

DEFINITION

abetes mellitus, nasogastric intubation, or recent antimicrobial therapy, who are frequently colonized with aerobic gram-negative rods); patients undergoing anesthesia; those with strokes, dementia, swallowing disorders; the frail elderly; and those receiving antacids or H2 blockers (but not sucralfate) • Hypoxic patients receiving concentrated O2 have diminished ciliary activity, encouraging aspiration • Causative organisms: 1. Anaerobes listed previously, although in many studies gramnegative aerobes (60%) and grampositive aerobes (20%) predominate 2. E. coli, P. aeruginosa, S. aureus, Klebsiella, Enterobacter, Serratia, and Proteus spp. H. influenzae, S. pneumoniae, Legionella, and Acinetobacter spp. (sporadic pneumonias) in two thirds of cases 3. Fungi, including Candida albicans, in fewer than 1%

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Pneumonia, Bacterial BASIC INFORMATION DEFINITION Bacterial pneumonia is an infection involving the lung parenchyma ICD-9CM CODES 486.0 Pneumonia, acute 507.0 Pneumonia, aspiration 482.9 Pneumonia, bacterial 481 Pneumonia, pneumococcal 482.1 Pneumonia, Pseudomonas 482.4 Pneumonia, staphylococcal 428.0 Pneumonia, Klebsiella 482.2 Pneumonia, Haemophilus influenzae

EPIDEMIOLOGY & DEMOGRAPHICS • Incidence of community-acquired pneumonia is 1/100 persons. • Incidence of nosocomial pneumonia is 8 cases/1000 persons/yr. • Primary care physicians see an average of 10 cases of pneumonia annually. • Hospitalization rate for pneumonia is 15% to 20%. • Most cases of pneumonia occur in the winter and in elderly patients.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Fever, tachypnea, chills, tachycardia, cough • Presentation varies with the cause of pneumonia, the patient’s age, and the clinical situation: 1. Patients with streptococcal pneumonia usually present with high fever, shaking chills, pleuritic chest pain, cough, and copious production of purulent sputum. 2. Elderly or immunocompromised hosts may initially present with only minimal symptoms (e.g., lowgrade fever, confusion); respiratory and nonrespiratory symptoms are less commonly reported by older patients with pneumonia. 3. Generally, auscultation reveals crackles and diminished breath sounds. 4. Percussion dullness is present if the patient has pleural effusion.

ETIOLOGY • Streptococcus pneumoniae • Haemophilus influenzae • Legionella pneumophila (1% to 5% of adult pneumonias) • Klebsiella, Pseudomonas, E. coli • Staphylococcus aureus • Pneumococcal infection is responsible for 50% to 75% of community-acquired pneumonias, whereas gram-negative organisms cause 80% of nosocomial pneumonias

• Predisposing factors are: 1. COPD: H. influenzae, S. pneumoniae, Legionella 2. Seizures: aspiration pneumonia 3. Compromised hosts: Legionella, gram-negative organisms 4. Alcoholism: Klebsiella, S. pneumoniae, H. influenzae 5. HIV: S. pneumoniae 6. IV drug addicts with right-sided bacterial endocarditis: S. aureus

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • • • • • •

Exacerbation of chronic bronchitis Pulmonary embolism or infarction Lung neoplasm Bronchiolitis Sarcoidosis Hypersensitivity pneumonitis Pulmonary edema Drug-induced lung injury Viral pneumonias Fungal pneumonias Parasitic pneumonias Atypical pneumonia Tuberculosis

WORKUP Laboratory evaluation and chest x-ray examination

LABORATORY TESTS • In hospitalized patients, attempt to obtain an adequate sputum specimen for Gram stain and cultures. 1. An expectorated sputum sample is often inadequate because of many false positive results (secondary to contamination from oral flora) and many false negative results; a specimen may be considered adequate if the Gram stain shows 25 PMNs and 10 epithelial cells per lowpower field. 2. Aerosol induction with hypertonic saline solution (3% to 10%) may increase the diagnostic yield of sputum. 3. The use of fiberoptic bronchoscopy to obtain a sputum sample is generally reserved for critically ill patients responding poorly to initial antimicrobial therapy. 4. Gram stain of sputum may reveal the following: Lancet-shaped, gram-positive cocci indicate streptococcal pneumonia. Pleomorphic, small coccobacillary, gram-negative organisms indicate H. influenzae. Encapsulated gram-negative bacilli: K. pneumoniae • WBC count is elevated, usually with left shift

• Blood cultures: positive in approximately 20% of cases of pneumococcal pneumonia • Pulse oximetry or ABGs: hypoxemia with partial pressure of oxygen 60 mm Hg while the patient is breathing room air is a standard criterion for hospital admission • Direct immunofluorescent examination of sputum when suspecting Legionella (e.g., direct fluorescent antibody [DFA] stain is a highly specific and rapid test for detecting legionellae in clinical specimen) • Serologic testing for HIV in selected patients

IMAGING STUDIES Chest x-ray: findings vary with the stage and type of pneumonia and the hydration of the patient (Fig. 1-32). • Classically, pneumococcal pneumonia presents with a segmental lobe infiltrate. • Diffuse infiltrates on chest x-ray can be seen with L. pneumophila, M. pneumoniae, viral pneumonias, P. carinii, miliary TB, aspiration, aspergillosis. • An initial chest x-ray is also useful to rule out the presence of any complications (pneumothorax, empyema, abscesses).

TREATMENT NONPHARMACOLOGIC THERAPY • Avoidance of tobacco use • Oxygen to maintain partial oxygen pressure in arterial blood 60 mm Hg • IV hydration, correction of dehydration • Assisted ventilation in patients with significant respiratory failure

ACUTE GENERAL Rx • Initial antibiotic therapy should be based on clinical, radiographic, and laboratory evaluation. • Macrolides (azithromycin or clarithromycin) or levofloxacin is recommended for empirical out-patient treatment of community-acquired pneumonia; cefotaxime or a betalactam/beta-lactamase inhibitor can be added in patients with more severe presentation who insist on out-patient therapy. Duration of treatment ranges from 7 to 14 days. • In the hospital setting, patients admitted to the general ward can be treated empirically with a second- or thirdgeneration cephalosporin (ceftriaxone, ceftizoxime, cefotaxime, or cefuroxime) plus a macrolide (azithromycin or clarithromycin) or doxycycline. An antipseudomonal quinolone (levo-

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Pneumonia, Bacterial

Parapneumonic effusion-empyema can be managed with chest tube placement for drainage. Instillation of fibrinolytic agents (streptokinase, urokinase) via the chest tube may be necessary in resistant cases.

DISPOSITION • Most patients respond well to antibiotic therapy. • Indications for hospital admission are: 1. Hypoxemia (oxygen saturation 90% while patient is breathing room air) 2. Hemodynamic instability

PEARLS & CONSIDERATIONS

• Pulmonary embolism • Immunologic or idiopathic: Wegener’s granulomatosis, pulmonary eosinophilic syndromes, SLE • Drug toxicity (e.g., amiodarone)

SUGGESTED READINGS Davidson R et al: Resistance to levofloxacin and failure of treatment of pneumococcal pneumonia, N Engl J Med 346:747, 2002. Halm EA, Teirstein AS: Management of community-acquired pneumonia, N Engl J Med 347:2039, 2002. AUTHOR: FRED F. FERRI, M.D.

COMMENTS Causes of slowly resolving or nonresolving pneumonia: • Difficult to treat infections: viral pneumonia, Legionella, pneumococci, or staphylococci with impaired host response, TB, fungi • Neoplasm: lung, lymphoma, metastasis • CHF

A

B

FIGURE 1-32 A, PA and, B, lateral chest radiographs reveal right upper lobe pneumonia and patchy left lower lobe infiltrate. A variety of organisms can produce this pattern, including S. pneumoniae and H. influenzae. (From Marx J [ed]: Rosen’s Emergency medicine, ed 5, St Louis, 2003, Mosby.)

DISEASES AND DISORDERS

CHRONIC Rx

3. Inability to tolerate medications 4. Active co-existing condition requiring hospitalization • The decision to treat a nursing home patient at the facility or in the hospital depends on the condition of the patient (e.g., stable vital signs and ability to maintain O2 sat above 90%), the resources available at the nursing home (e.g., staffing, access to IV therapy), and the overall plan of care for the patient (e.g., advance directives).

Section I

floxacin, moxifloxacin, or gatifloxacin) may be substituted in place of the macrolide or doxycycline. • In hospitalized patients at risk for P. aeruginosa infection, empirical treatment should consist of an antipseudomonal beta-lactam (cefepime or piperacillin-tazobactam) plus an aminoglycoside plus an antipseudomonal quinolone or macrolide.

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Polymyalgia Rheumatica BASIC INFORMATION DEFINITION Polymyalgia rheumatica is a disorder of unknown cause affecting older patients. It is characterized by shoulder and hip stiffness and an elevated erythrocyte sedimentation rate (ESR).

SYNONYMS Anarthritic rheumatoid syndrome ICD-9CM CODES 725.0 Polymyalgia rheumatica

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 1 case/135 persons 50 yr old PREDOMINANT SEX: Female:male ratio of 2:1 PREDOMINANT AGE: Average age at onset: 70 yr

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Symptoms are frequently of sudden onset but are often present for months before the diagnosis is made. • Neck, shoulder, low back, and thigh pain are common complaints. • Morning stiffness lasting 2 to 3 hr is typical, and patients often have difficulty getting out of bed. • Malaise, weight loss, depression, and a low-grade fever are common constitutional symptoms and may suggest systemic inflammation.

TABLE 1-12

• Physical findings are usually limited. Synovitis may be present in peripheral joints and may also be responsible for the proximal girdle symptoms in spite of the fact that they appear to be “muscular” in nature. • Mild soft tissue tenderness may be present. • Distal extremity manifestations (knee, wrist, metacarpophalangeal joints) may occur in 25% to 45% of patients. • The temporal arteries should be carefully examined because of the strong relation of polymyalgia rheumatica with temporal or giant cell arteritis.

ETIOLOGY Unknown

DIAGNOSIS DIFFERENTIAL DIAGNOSIS (Table 1-12) • Rheumatoid arthritis: rheumatoid factor is negative in polymyalgia. • Polymyositis: enzyme studies are negative in polymyalgia. • Fibromyalgia

WORKUP The diagnosis of polymyalgia rheumatica is suggested by the following findings: • Pain and stiffness of pectoral and pelvic musculature • Patient 50 yr old • Morning stiffness 1 hr • Normal motor strength • Symptoms for at least 4 to 6 wk

• Elevated ESR (45) • Rapid clinical response to low-dose corticosteroid therapy

LABORATORY TESTS • CBC, ESR, and rheumatoid factor should be performed. • Mild anemia may be present.

TREATMENT ACUTE GENERAL Rx • Prednisone 10 to 20 mg/day is given. The response is often so dramatic that it can be used to confirm the diagnosis. Improvement is usually noted within 24 to 48 hr. Generally, if the initial prednisone dose is 20 mg/day, reduce by 2.5 mg every wk to 10 mg/day, then by 1 mg/day every month if tolerated. • Steroids are gradually tapered over the next few weeks as soon as symptoms permit, but small doses (5 mg/day) may be needed for 2 yr. • NSAIDs may be tried in mild cases. • Physical therapy is usually unnecessary.

PEARLS & CONSIDERATIONS COMMENTS The prognosis is generally favorable. Relapse occasionally occurs in several years, but again responds well to prednisone.

Differential Features in Polymyalgia Rheumatica and Similar Disorders

Signs/Symptoms Morning stiffness 30 min Headache and/or scalp tenderness Pain with active joint movement Tender joints Swollen joints Muscle weakness Normochromic anemia Elevated erythrocyte sedimentation rate Elevated serum creatine kinase Serum rheumatoid factor Distinct electromyographic abnormality Response to nonsteroidal antiinflammatory drug

Polymyalgia Rheumatica

Giant Cell Arteritis

Rheumatoid Arthritis

Dermatomyositis

Fibromyalgia

 0    †   0 0 0 

  0 0  0   0 0 0 0

* 0 * *  *   0 70% 0 

 0 0 0 0  0   0  0

Variable Variable Inconstant Tender spots 0 0 0 0 0 0 0 0

From Goldman L, Ausiello D, (eds): Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders. 0, Absent; , present; , present in minority of cases. *Associated with affected joints † Pain inhibits movement. Disuse atrophy may occur.

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Polymyalgia Rheumatica SUGGESTED READINGS

Meskimen S, Cook TD, Blake RL: Management of giant cell arteritis and polymyalgia rheumatica, Am Fam Physician 61:2061, 2000. Salvarani C et al: Polymyalgia rheumatica and giant-cell arteritis, N Engl J Med 347:261, 2002. AUTHOR: LONNIE R. MERCIER, M.D.

DISEASES AND DISORDERS

De Jager JP: Polymyalgia rheumatica and giant cell arteritis: avoiding management traps, Aust Fam Physician 30:643, 2001. Mandell BF: Polymyalgia rheumatica: clinical presentation is key to diagnosis and treatment, Cleve Clin J Med 71(6):489, 2004. Marti J, Anton E: Polymyalgia rheumatica complication influenza vaccination, J Am Geriatr Soc 52(8):1412, 2004.

Section I

Cimmino MA, Macchioni P et al: Pulse steroid treatment of polymyalgia rheumatica, Clin Exp Rheumatol 22(3):381, 2004. Clough JD: Polymyalgia rheumatica: not well understood, but important to consider, Cleve Clin J Med 71(6):446, 2004. Cohen MD, Abril A: Polymyalgia rheumatica revisited, Bull Rheum Dis 50:1, 2001.

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Prostate Cancer BASIC INFORMATION DEFINITION Prostate cancer is a neoplasm involving the prostate; various classifications have been developed to evaluate malignancy potential and prognosis: • The degree of malignancy varies with the stage: Stage A: Confined to the prostate, no nodule palpable Stage B: Palpable nodule confined to the gland Stage C: Local extension Stage D: Regional lymph nodes or distant metastases • In the Gleason classification, histologic patterns are independently assigned numbers 1 to 5 (best to least differentiated). These numbers are added to give a total tumor score: 1. Prognosis is generally good if score is 5. 2. Score of 6, 7, or 8 carries an intermediate prognosis. 3. Score 9 correlates with anaplastic lesions with poor prognosis. ICD-9CM CODES 185 Malignant neoplasm of prostate

EPIDEMIOLOGY & DEMOGRAPHICS • Prostate cancer has surpassed lung cancer as the most common nonskin cancer in men. • More than 100,000 cases are diagnosed yearly, and nearly 30,000 men die from prostate cancer each year (second leading cause of death from cancer in U.S. men). • Incidence of prostate cancer increases with age: 80% of new cases are diagnosed in patients 65 yr. • Average age at time of diagnosis is 72 yr. • Blacks in the U.S. have the highest incidence of prostate cancer in the world (one in every nine males). • Incidence is low in Asians. • Approximately 9% of all prostate cancers may be familial.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Generally silent disease until it reaches advanced stages. • Bone pain and pathologic fractures may be initial symptoms of prostate cancer. • Local growth can cause symptoms of urinary outflow obstruction. • Digital rectal examination (DRE) may reveal an area of increased firmness. An asymmetric prostate is also suspicious for cancer. 10% of patients will have a negative DRE.

• Prostate may be hard, fixed, with extension of tumor to the seminal vesicles in advanced stages.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Benign prostatic hypertrophy • Prostatitis • Prostate stones

LABORATORY TESTS • Measurement of PSA is useful in early diagnosis of prostate cancer (screening) and in monitoring response to therapy as well as an early sign of recurrence. Normal PSA is found in 20% of patients with prostate cancer (lack of sensitivity), whereas only 20% of men with PSA levels between 4 ng/ml and 10 ng/ml have prostate cancer (lack of specificity). PSA 10 ng/ml is associated with more advanced prostate cancer. The American Cancer Society recommends offering the PSA test and digital rectal examination yearly to men 50 years or older who have a life expectancy of at least 10 years. Earlier testing, starting at age 40, is recommended for men at high risk (e.g., African Americans and men with a family history of prostate cancer). The most common causes of false-positive PSA are BPH, prostatitis, and ejaculation within 48 hours of testing. Rectal exam causes only a minimal rise in PSA (of 0.2 ng/ml over baseline). Finasteride and dutasteride reduce PSA values by 50%. • Ideally, prostate cancer should be diagnosed when the PSA is between 4 and 10 mg/ml (between 2.6 and 10 ng/ml for African American men). Unfortunately, in this range of PSA, many men do not have cancer (falsepositive test) and prostate biopsies are invasive, uncomfortable, and themselves subject to false-negative results from sampling. To cope with this, many clinicians use the PSA velocity concept. In the absence of cancer, the PSA rises slowly over time in a linear pattern. With cancer, the rate of PSA increase can be exponential. Instead of repeating the PSA annually when the result is between 4 and 10 mg/ml, it is repeated every 3 months until a pattern of increase can be ascertained. Also, PSA should not rise by more than 0.85 mg/ ml per year and should take 4 years to double in the absence of cancer. • The use of serum-free PSA for prostate screening has been proposed by some urologists as a means to decrease unwarranted biopsies without missing a significant number of prostate cancers. This approach is based on the higher

•

•

• •

free PSA in men with benign prostatic hyperplasia and the higher proteinbound PSA levels in men with prostate cancer. For example, in men with total PSA levels of 4 to 10 ng/ml, the cancer probability is 0.25, but if the percentage of free PSA is 10%, the probability of cancer increases to 0.45, and if the percentage of free PSA is 25%, the probability of cancer decreases to 8%. If the PSA is 2 mg/ml in non–African American men, the frequency of screening can drop from yearly to every 2 years. If the DRE reveals a suspicious prostate for cancer, referral to a urologist for prostate biopsy is appropriate regardless of PSA level. Prostatic acid phosphatase (PAP) can be used for evaluation of nonlocalized disease. Transrectal biopsy and fine-needle aspiration of prostate can confirm the diagnosis.

IMAGING STUDIES • According to the AUA screening, transrectal ultrasonography adds little to the combination of PSA and digital rectal examination. • Bone scan is useful to evaluate bone metastasis. However, according to the American Urological Association (AUA), the routine use of bone scanning is not required for staging of prostate cancer in asymptomatic men with clinically localized cancer if the PSA level is 20 ng/ml. • CT scan, MRI, and transrectal ultrasonography may be useful in selected patients to assess extent of prostate cancer. CT and MRI imaging are generally not indicated for cancer staging in men with clinically localized cancer and PSA 25 ng/ml. With regard to pelvic lymph node dissection in staging, the AUA states that it may not be required in patients with PSA levels 10 ng/ml and when PSA level is 20 ng/ml and the Gleason score is 6. High-resolution MRI with magnetic nanoparticles has been used for the detection of small and otherwise undetectable lymph node metastases in patients with prostate cancer.

TREATMENT NONPHARMACOLOGIC THERAPY Watchful waiting is reasonable in patients with early stage (T-IA) and projected life expectancy 10 yr, or in patients with focal and moderately differentiated carcinoma.

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Prostate Cancer ACUTE GENERAL Rx

CHRONIC Rx • Patients should be monitored at 3- to 6-mo intervals with clinical examination, and PSA for the first year, then every 6 mo for the second year, then yearly if stable. For patients who have undergone radical prostatectomy, a rising PSA level suggests evidence of residual or recurrent prostate cancer. • Chest x-ray examination, bone scan should be performed yearly or sooner if patient develops symptoms.

DISPOSITION • Prognosis varies with the stage of the disease and the Gleason classification (see Definition & Classification). • The ploidy of the tumor also has prognostic value: prognosis is better with diploid tumor cells, worse with aneuploid tumor cells. • For Stage A tumors, the extended 10yr, disease-specific survival is similar for patients with prostatectomy (94%), radiotherapy (90%), and conservative management (93%); survival rate is better with surgery than with radio-

therapy or conservative management in patients with Stage B or C localized prostate cancer. • Expression of the gene EZH2 has been identified as an important factor in the determination of the aggressiveness of prostate cancer. A recent study revealed that expression of the EZH2 gene may be a better predictor of clinical failure than Gleason score, tumor stage, or surgical margin status. Testing for EZH2 protein in prostate cancer tissue may be useful to determine prognosis and direct treatment.

SUGGESTED READINGS Gann PH et al: Strategies combining total and percent free prostate specific antigen for detecting prostate cancer: a perspective evaluation, J Urol 167:2427, 2002. Harisinghani MG et al: Noninvasive detection of clinically occult lymph-node metastases in prostate cancer, N Engl J Med 348:2491, 2003. Homberg L et al: A randomized trial comparing radical prostatectomy with watchful waiting in early prostate cancer, N Engl J Med 347:781, 2002. Makinen T et al: Family history and prostate cancer screening with prostate specific antigen, J Clin Oncol 20:2658, 2002. Nelson WG: Prostate cancer, N Engl J Med 349:366, 2003. Steineck G et al: Quality of life after radical prostatectomy or watchful waiting, N Engl J Med 347:790, 2002. Varambally S et al: The polycarb group protein EZH2 is involved in progression of prostate cancer, Nature 419:624, 2002. AUTHORS: FRED F. FERRI, M.D., and TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

metastatic prostate cancer. Adjuvant treatment with GnRH agonists (goserelin leuprolide, or triptorelin) plus antiandrogens (flutamide, bicalutamide, or nilutamide), when started simultaneously with external irradiation, improves local control and survival in patients with locally advanced prostate cancer. Bisphosphonates inhibit osteoclast-mediated bone resorption and prevent bone loss in the hip and lumbar spine in men receiving treatment for prostate cancer with a GnRH.

Section I

• Therapeutic approach varies with the following: 1. Stage of the tumor 2. Patient’s life expectancy 3. General medical condition 4. Patient’s treatment preference (e.g., patient may be opposed to orchiectomy) • The optimal treatment of clinically localized prostate cancer is unclear. 1. Radical prostatectomy is generally performed in patients with localized prostate cancer and life expectancy 10 yr. 2. Radiation therapy (external beam irradiation or implantation of radioactive pellets [seeds]) represents an alternative in patients with localized prostate cancer, especially poor surgical candidates or patients with a high-grade malignancy. 3. Watchful waiting is reasonable in patients who are too old or too ill to survive longer than 10 yr. If the cancer progresses to the point where it becomes symptomatic, palliation can be attempted with several methods. • Patients with advanced disease and projected life expectancy 10 yr are candidates for radiation therapy and hormonal therapy (DES, LHRH analogs, antiandrogens, bilateral orchiectomy). • Recommended treatment of patients with regional metastatic prostate cancer with projected life expectancy 10 yr includes radical prostatectomy, radiation therapy, hormonal therapy. • Androgen-deprivation therapy with a gonadotropin-releasing hormone agonist is the mainstay of treatment for

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Prostatitis BASIC INFORMATION DEFINITION Prostatitis refers to inflammation of the prostate gland. There are four major categories: • Acute bacterial prostatitis • Chronic bacterial prostatitis • Nonbacterial prostatitis • Prostatodynia ICD-9CM CODES 601.0 Prostatitis (acute) 601.1 Prostatitis (chronic) 099.54 Prostatitis (chlamydial)

EPIDEMIOLOGY & DEMOGRAPHICS • 50% of men experience symptoms of prostatitis in their lifetime • Acute bacterial prostatitis is uncommon • The relative prevalence of the other three entities among men with inflammatory prostatic symptoms is: 1. 5% to 10% chronic bacterial prostatitis 2. 10% to 65% nonbacterial prostatitis 3. 30% to 80% prostatodynia The figures are imprecise because nonbacterial prostatitis and prostatodynia are very difficult to differentiate.

PHYSICAL FINDINGS & CLINICAL PRESENTATION ACUTE BACTERIAL PROSTATITIS: • Sudden or rapidly progressive onset of: 1. Dysuria 2. Frequency 3. Urgency 4. Nocturia 5. Perineal pain that may radiate to the back, the rectum, or the penis • Hematuria or a purulent urethral discharge may occur. • Occasionally urinary retention complicates the course. • Fever, chills, and signs of sepsis can also be part of the clinical picture. • On rectal examination the prostate is typically tender. CHRONIC BACTERIAL PROSTATITIS: • May be asymptomatic when the infection is confined to the prostate • May present as an increase in severity of baseline symptoms of benign prostatic hypertrophy • When cystitis is also present, urinary frequency, urgency, and burning may be reported • Hematuria may be a presenting complaint • In elderly men, new onset of urinary incontinence may be noted

NONBACTERIAL PROSTATITIS AND PROSTATODYNIA: • Present similarly with symptoms of bladder irritation (urinary frequency, urgency, dysuria, increase in nocturia episodes) and perineal discomfort • The symptoms can be of variable severity, but tend to be more bothersome in prostatodynia

ETIOLOGY ACUTE BACTERIAL PROSTATITIS: • Acute usually gram-negative infection of the prostate gland 1. Generally associated with cystitis 2. Resulting from the ascent of bacteria in the urethra • Occasionally the route of infection is hematogenous or a lymphatogenous spread of rectal bacteria • The condition is more likely to occur in young or middle-aged men CHRONIC BACTERIAL PROSTATITIS: • Often asymptomatic • Exacerbation of symptoms of benign prostatic hypertrophy caused by the same mechanism as in acute bacterial prostatitis NONBACTERIAL PROSTATITIS: • Refers to symptoms of prostatic inflammation associated with the presence of WBCs in prostatic secretions with no identifiable bacterial organism • Chlamydia infection may be etiologically implicated in some cases PROSTATODYNIA: • Refers to symptoms of prostatic inflammation with no or few WBCs in the prostatic secretion • Spasm in the bladder neck or urethra is felt to be the cause of symptoms

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Benign prostatic hypertrophy with lower urinary tract symptoms • Prostate cancer • Also see differential diagnosis of hematuria

WORKUP • Rectal examination 1. Tender prostate most suggestive of acute bacterial prostatitis 2. Enlarged prostate common in chronic bacterial prostatitis 3. Normal prostate is consistent with chronic bacterial and nonbacterial prostatitis and is typical in prostatodynia • Expression of prostatic secretions (EPS) by prostate massage is contraindicated in acute bacterial prostatitis but is appropriate in the other three situations

LABORATORY TESTS • Urinalysis • Urine culture and sensitivity • Bacterial localization studies can be performed but are cumbersome and impractical in most clinical settings • Cell count and culture of expressed prostatic secretions • The yield of a urine culture may be increased if the specimen is obtained after a prostatic massage • PSA is not used to diagnose prostatitis; however, a rapid rise over baseline should raise the possibility of prostatitis even in the absence of symptoms. In such cases, a follow-up PSA after treatment of prostatitis is appropriate • CBC and blood cultures if fever, chills, or signs of sepsis exist • If hematuria is present, a workup to rule out a urologic malignancy should be considered if the hematuria does not clear after treatment of prostatitis

TREATMENT ACUTE BACTERIAL PROSTATITIS: Culture guided antibiotic therapy for 4 wk (beginning with a few days of intravenous antibiotics if the infection is serious or if the patient is bacteremic). CHRONIC BACTERIAL PROSTATITIS: • Trimethoprim-sulfamethoxazole is first-line choice for 4 wk if the organism is sensitive. • Second-line choice for treatment failure or organisms resistant to TMP-SMX is with a fluoroquinolone. • Patient with refractory infection or with multiple relapses may be offered long-term suppressive therapy. NONBACTERIAL PROSTATITIS AND PROSTATODYNIA: • No specific treatment • Antibiotics are not effective • A trial of treatment with an -adrenergic blocker (terazosin, doxazosin, or tamsulosin) may be considered • Any underlying bladder pathology should be ruled out by cystoscopy and treated if identified

SUGGESTED READINGS Fowler JE: Prostatitis. In Gillenwater JY et al (eds): Adult and Pediatric Urology, St Louis, 1996, Mosby. McNaughton Collins M, MacDonald R, Wilt TJ: Diagnosis and treatment of chronic abacterial prostatitis: a systemic review, Ann Intern Med 133:367, 2000. AUTHOR: TOM J. WACHTEL, M.D.

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Pseudogout BASIC INFORMATION

SYNONYMS Calcium pyrophosphate dehydrate crystal deposition disease (CPDD) Chondrocalcinosis Pyrophosphate arthropathy ICD-9CM CODES 275.4 Chondrocalcinosis

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: • Uncertain • Probably similar to gout (3/1000 persons) • Chondrocalcinosis is present in 20% of all people at age 80 yr, but most are asymptomatic PREDOMINANT SEX: Female:male ratio of approximately 1.5:1 PREVALENT AGE: 60 to 70 yr at onset

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Symptoms are similar to those of gouty arthritis with acute attacks and chronic arthritis • Knee joint is most commonly affected • Swelling, stiffness, and increased heat in affected joint

ETIOLOGY • Unknown • Often associated with various medical conditions, including hyperparathyroidism and amyloidosis

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Gouty arthritis • Rheumatoid arthritis • Osteoarthritis • Neuropathic joint Section II describes the differential diagnosis of acute monoarticular and oligoarticular arthritis and crystal-induced arthritides. An algorithm for evaluation of arthralgia is described in Section III, “Arthralgia Limited to One or Few Joints.”

Crystal analysis of the synovial fluid aspirate to reveal rhomboid calcium pyrophosphate crystals

IMAGING STUDIES Plain radiographs to reveal the following: • Stippled calcification in bands running parallel to the subchondral bone margins • Crystal deposition in menisci, synovium, and ligament tissue; triangular wrist cartilage and symphysis pubis are often affected

TREATMENT NONPHARMACOLOGIC THERAPY General measures such as heat, rest, and elevation as needed

ACUTE GENERAL Rx • NSAIDs (as for gout) • Colchicine • Aspiration/steroid injection

DISPOSITION Structural joint damage may occasionally occur, requiring arthroplasty in rare cases.

REFERRAL For orthopedic consultation for destructive joint changes

PEARLS & CONSIDERATIONS COMMENTS As with gout, acute attacks may be triggered by various surgical or medical events.

SUGGESTED READINGS Agudelo CA, Wise CM: Crystal-associated arthritis in the elderly, Rheum Dis Clin North Am 26:527, 2000. Canhao H et al: Cross-sectional study of 50 patients with calcium pyrophosphate dihydrate crystal arthropathy, Clin Rheumatol 20:119, 2001. Halverson PB, Derfus BA: Calcium crystal-induced inflammation, Curr Opin Rheumatol 13:221, 2001. Mader B: Calcium pyrophosphate dihydrate deposition disease of the wrist, Clin Rheumatol 23(1):95, 2004. Rosenthal AK: Crystal arthropathies and other unpopular rheumatic diseases, Curr Opin Rheumatol 16(3):262, 2004. Sagarin MJ: Pseudogout, Emerg Med 18:373, 2000. AUTHOR: LONNIE R. MERCIER, M.D.

DISEASES AND DISORDERS

Pseudogout is one of the clinical patterns associated with a crystal-induced synovitis resulting from the deposition of calcium pyrophosphate dehydrate (CPPD) crystals in joint hyaline and fibrocartilage. The cartilage deposition is termed chondrocalcinosis.

LABORATORY TESTS

• Variable clinical presentation • Diagnosis dependent on the identification of CPPD crystals • The American Rheumatism Association revised diagnostic criteria for CPPD crystal deposition disease (pseudogout) are often used: 1. Criteria I. Demonstration of CPPD crystals (obtained by biopsy, necroscopy, or aspirated synovial fluid) by definitive means (e.g., characteristic “fingerprint” by x-ray diffraction powder pattern or by chemical analysis) II. (a) Identification of monoclinic and/or triclinic crystals showing either no or only a weakly positive birefringence by compensated polarized light microscopy (b) Presence of typical calcifications in roentgenograms III. (a) Acute arthritis, especially of knees or other large joints, with or without concomitant hyperuricemia (b) Chronic arthritis, especially of knees, hips, wrists, carpus, elbow, shoulder, and metacarpophalangeal joints, especially if accompanied by acute exacerbations; the following features are helpful in differentiating chronic arthritis from osteoarthritis: 1. Uncommon site—for example, wrist, MCP, elbow, shoulder 2. Appearance of lesion radiologically—for example, radiocarpal or patellofemoral joint space narrowing, especially if isolated (patella “wrapped around” the femur) 3. Subchondral cyst formation 4. Severity of degeneration— progressive, with subchondral bony collapse (microfractures), and fragmentation, with formation of intraarticular radiodense bodies 5. Osteophyte formation— variable and inconstant 6. Tendon calcifications, especially Achilles, triceps, obturators 2. Categories Definite—Criteria I or II(a) plus (b) must be fulfilled. Probable—Criteria II(a) or II(b) must be fulfilled. Possible—Criteria III(a) or (b) should alert the clinician to the possibility of underlying CPPD deposition.

Section I

DEFINITION

WORKUP

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Pseudomembranous Colitis BASIC INFORMATION DEFINITION Pseudomembranous colitis is the occurrence of diarrhea and bowel inflammation associated with antibiotic use.

SYNONYMS Antibiotic-induced colitis Clostridium dificile colitis ICD-9CM CODES 008.45 Clostridium difficile, pseudomembranous colitis

EPIDEMIOLOGY & DEMOGRAPHICS • Cephalosporins are the most frequent offending agent in pseudomembranous colitis because of their high rates of use. • The antibiotic with the highest incidence is clindamycin (10% incidence of pseudomembranous colitis with its use). • Clostridium difficile causes more than 250,000 cases of diarrhea and colitis in the U.S. every year.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Abdominal tenderness (generalized or lower abdominal) • Fever • In patients with prolonged diarrhea, poor skin turgor, dry mucous membranes, and other signs of dehydration may be present

ETIOLOGY Risk factors for C. difficile (the major identifiable agent of antibiotic-induced diarrhea and colitis): • Administration of antibiotics: can occur with any antibiotic, but occurs most frequently with clindamycin, ampicillin, and cephalosporins • Prolonged hospitalization • Advanced age • Abdominal surgery • Hospitalized, tube-fed patients are at risk for C. difficile–associated diarrhea. Clinicians should consider testing for C. difficile in tube-fed patients with diarrhea unrelated to the feeding solution

• IBD • Celiac sprue • Irritable bowel syndrome

WORKUP • All patients with diarrhea accompanied by current or recent antibiotic use should be tested for C. difficile (see “Laboratory Tests”). • Sigmoidoscopy (without cleansing enema) may be necessary when the clinical and laboratory diagnosis is inconclusive and the diarrhea persists. • In antibiotic-induced pseudomembranous colitis, the sigmoidoscopy often reveals raised white-yellow exudative plaques adherent to the colonic mucosa (Fig. 1-33).

LABORATORY TESTS • C. difficile toxin can be detected by cytotoxin tissue-culture assay (gold standard for identifying C. difficile toxin in stool specimen) and by enzyme-linked immunoabsorbent assay (ELISA) for C. difficile toxins A and B. The latter is used most widely in the clinical setting. It has a sensitivity of 85% and a specificity of 100%. • Fecal leukocytes (assessed by microscopy or lactoferrin assay) are generally present in stool samples. • CBC usually reveals leukocytosis.

IMAGING STUDIES Abdominal film (flat plate and upright) is useful in patients with abdominal pain or evidence of obstruction on physical examination.

TREATMENT NONPHARMACOLOGIC THERAPY • Discontinue offending antibiotic • Fluid hydration and correct electrolyte abnormalities

ACUTE GENERAL Rx • Metronidazole 500 mg PO tid for 10 to 14 days • Vancomycin 125 mg PO qid for 10 to 14 days in cases resistant to metronidazole • Cholestyramine 4 g PO qid for 10 days in addition to metronidazole to control severe diarrhea (avoid use with vancomycin) • When parenteral therapy is necessary (e.g., patient with paralytic ileus), IV metronidazole 500 mg qid can be used. It can also be supplemented with vancomycin 500 mg via NG tube or enema

CHRONIC Rx Judicious future use of antibiotics to prevent recurrences (e.g., avoid prolonged antibiotic therapy)

DISPOSITION Most patients recover completely with appropriate therapy. Fever resolves within 48 hr and diarrhea within 4 to 5 days. Mortality exceeds 10% in untreated patients.

REFERRAL Hospital admission and IV hydration in severe cases

PEARLS & CONSIDERATIONS COMMENTS Possible complications of pseudomembranous colitis include dehydration, bowel perforation, toxic megacolon, electrolyte imbalance, and reactive arthritis.

SUGGESTED READINGS Bartlett JG: Antibiotic-associated diarrhea, N Engl J Med 346:334, 2002. Hurley BW, Nguyen CC: The spectrum of pseudomembranous enterocolitis and antibiotic-associated diarrhea, Arch Intern Med 162:2177, 2002. AUTHOR: FRED F. FERRI, M.D.

DIAGNOSIS The clinical signs of pseudomembranous colitis generally include diarrhea, fever, and abdominal cramps following use of antibiotics.

DIFFERENTIAL DIAGNOSIS • GI bacterial infections (e.g., Salmonella, Shigella, Campylobacter, Yersinia) • Enteric parasites (e.g., Cryptosporidium, Entamoeba histolytica)

FIGURE 1-33 Pseudomembranous plaques seen with colonoscopy in a patient with C. difficile-associated PMC. (From Gorbach SL: Infectious diseases, ed 2, Philadelphia, 1998, WB Saunders.)

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Pulmonary Embolism BASIC INFORMATION DEFINITION Pulmonary embolism (PE) refers to the lodging of a thrombus or other embolic material from a distant site in the pulmonary circulation. Pulmonary thromboembolism PE ICD-9CM CODES 415.1 Pulmonary embolism and infarction

EPIDEMIOLOGY & DEMOGRAPHICS • 650,000 cases of PE occur in the U.S. each year; 50,000 result in death (increased incidence in women and with advanced age). • More than 90% of pulmonary emboli originate in the deep venous system of the lower extremities. • Pulmonary thromboembolism is associated with 200,000 hospitalizations each year in the U.S. • 8% to 10% of victims of PE die within the first hour.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Most common symptom: dyspnea • Chest pain: may be nonpleuritic or pleuritic (infarction) • Syncope (massive PE) • Fever, diaphoresis, apprehension • Hemoptysis, cough • Evidence of DVT may be present (e.g., swelling and tenderness of extremities) • Cardiac examination: may reveal tachycardia, increased pulmonic component of S2, murmur of tricuspid insufficiency, right ventricular heave, right-sided S3 • Pulmonary examination: may demonstrate rales, localized wheezing, friction rub • Most common physical finding: tachypnea

ETIOLOGY • Thrombus, fat, or other foreign material • Risk factors for PE: 1. Prolonged immobilization 2. Postoperative state 3. Trauma to lower extremities 4. Estrogen-containing birth control pills 5. Prior history of DVT or PE 6. CHF 7. Visceral cancer (lung, pancreas, alimentary and genitourinary tracts) 8. Trauma, burns 9. Advanced age

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • • • • •

Myocardial infarction Pericarditis Pneumonia Pneumothorax Chest wall pain GI abnormalities (e.g., peptic ulcer, esophageal rupture, gastritis) CHF Pleuritis Anxiety disorder with hyperventilation Pericardial tamponade Dissection of aorta Asthma

WORKUP • Clinical assessment alone is insufficient to diagnose or rule out PE. It is also important to remember that no single noninvasive test has both high sensitivity and high specificity for PE. Consequently, in addition to clinical assessment, most patients will require several noninvasive tests or pulmonary angiography to diagnose PE. • Spiral CT of chest or lung scan may be diagnostic. Pulmonary angiogram (when indicated) will confirm the diagnosis. • Serial compressive duplex ultrasonography of lower extremities can be used in patients with “low-probability” lung scan and high clinical suspicion (see “Imaging Studies”). It is useful if positive, negative results do not exclude pulmonary embolism.

LABORATORY TESTS • ABGs generally reveal decreased Pao2 and Paco2 and increased pH; normal results do not rule out PE. • Alveolar-arteriolar (A-a) oxygen gradient, a measure of the difference in oxygen concentration between alveoli and arterial blood, is a more sensitive indicator of the alteration in oxygenation than Pao2; it can easily be calculated using the information from ABGs; a normal A-a gradient among patients without history of PE or DVT makes the diagnosis of PE unlikely. • Plasma D-dimer measurement: D-dimer assays by ELISA detect the presence of plasmin-mediated degradation prod-

IMAGING STUDIES • Chest x-ray may be normal; suggestive findings include elevated diaphragm, pleural effusion, dilation of pulmonary artery, infiltrate or consolidation, abrupt vessel cut-off, or atelectasis. A wedgeshaped consolidation in the middle and lower lobes is suggestive of a pulmonary infarction and is known as “Hampton’s hump.” • Lung scan (in patient with normal chest x-ray examination): 1. A normal lung scan rules out PE. 2. A ventilation-perfusion mismatch is suggestive of PE, and a lung scan interpretation of high probability is confirmatory. 3. If the clinical suspicion of PE is high and the lung scan is interpreted as low probability, moderate probability, or indeterminate, a pulmonary arteriogram is diagnostic; a positive arteriogram confirms diagnosis; a positive compressive duplex ultrasonography for DVT obviates the need for an arteriogram, because treatment with IV anticoagulants is indicated in these patients; the overall sensitivity of compressive ultrasonography for DVT in patients with PE is 29%, specificity

DISEASES AND DISORDERS

SYNONYMS

ucts of fibrin that contain cross-linked D fragments in the whole blood or plasma. A normal plasma D-dimer level is useful to exclude pulmonary embolism in patients with a nondiagnostic lung scan and a low pretest probability of PE. However, it cannot be used to “rule in” the diagnosis because it increases with many other disorders (e.g., metastatic cancer, trauma, sepsis, postoperative state). Plasma D-dimer can also be used in conjunction with lower-extremity compression ultrasonography in patients with indeterminate V/Q and spiral CT scans. Absence of DVT and presence of a normal D-dimer level in these settings generally rules out clinically significant pulmonary embolism. • Elevated cardiac troponin levels also occur in patients with pulmonary embolism because of right ventricular dilation and myocardial injury; therefore, PE should be considered in the differential diagnosis of all patients presenting with chest pain or dyspnea and elevated cardiac troponin levels. • ECG is abnormal in 85% of patients with acute PE. Frequent abnormalities are sinus tachycardia; nonspecific STsegment or T wave changes; S-I, Q-III, T-III pattern (10% of patients); S-I, S-II, S-III pattern; T wave inversion in V1 to V6; acute RBBB; new-onset atrial fibrillation; ST segment depression in lead II; right ventricular strain.

Section I

10. Obesity 11. Hematologic disease (e.g., antithrombin III deficiency, protein C deficiency, protein S deficiency, lupus anticoagulant, polycythemia vera, dysfibrinogenemia, paroxysmal nocturnal hemoglobinuria, factor V Leiden mutation, G20210A prothrombin mutation) 12. COPD, diabetes mellitus 13. Prolonged air travel

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Pulmonary Embolism 97%; adding ultrasonography in patients with a nondiagnostic lung scan prevents 9% of angiographies; however, this improvement in efficacy is achieved at the cost of unnecessary anticoagulant therapy in 26% of patients who have false-positive ultrasonography results. • Spiral CT is an excellent modality for diagnosing PE. It may be used in place of the lung scan and is favored in patients with baseline lung abnormalities on initial chest x-ray. It has the added advantage of detecting other pulmonary pathology that can mimic pulmonary embolism. • Angiography: pulmonary angiography is the gold standard; however, it is invasive, expensive, and not readily available in some clinical settings. False-positive pulmonary angiograms may result from mediastinal disorders such as radiation fibrosis and tumors. CT angiography is an accurate, noninvasive tool in the diagnosis of PE at the main, lobar, and segmental pulmonary artery levels. A major advantage of CT angiography over standard pulmonary angiography is its ability to diagnose intrathoracic disease other than PE that may account for the patient’s clinical picture. It is also less invasive, less costly, and more widely available. Its major shortcoming is its poor sensitivity for subsegmental emboli. Gadoliniumenhanced magnetic resonance angiography of the pulmonary arteries has a moderate sensitivity and high specificity for the diagnosis of PE; MRA is best reserved for selected patients when CT scan and/or lung scan are inconclusive and the risk of pulmonary angiography is high.

TREATMENT NONPHARMACOLOGIC THERAPY Correction of risk factors (see “Etiology”) to prevent future PE

ACUTE GENERAL Rx • Heparin by continuous infusion for at least 5 days; many experts recommend a larger initial IV heparin bolus (15,000 to 20,000 U) to block platelet aggregation and thrombi and subsequent release of vasoconstrictive substances. • Thrombolytic agents (urokinase, tPA, streptokinase): provide rapid resolu-

tion of clots; thrombolytic agents are the treatment of choice in patients with massive PE who are hemodynamically unstable and with no contraindication to their use. The use of thrombolytic agents in the treatment of hemodynamically stable patients with acute submassive pulmonary embolism remains controversial. Use of the thrombolytic agents alteplase (100 mg IV over a 2-hr period) in normotensive patients with moderate or severe right ventricular dysfunction identified by echocardiography has been advocated by some physicians. Use of alteplase in conjunction with heparin has been shown to improve the clinical course of stable patients who have acute submassive PE without internal bleeding. Additional studies are needed to confirm these findings before recommending routine use of this therapeutic approach. • Long-term treatment is generally carried out with warfarin therapy started on day 1 or 2 and given in a dose to maintain the INR at 2 to 3. • If thrombolytics and anticoagulants are contraindicated (e.g., GI bleeding, recent CNS surgery, recent trauma) or if the patient continues to have recurrent PE despite anticoagulation therapy, vena caval interruption is indicated by transvenous placement of a Greenfield vena caval filter. • Acute pulmonary artery embolectomy may be indicated in a patient with massive pulmonary emboli and refractory hypotension.

CHRONIC Rx Elimination of risk factors (see “Etiology”) and monitoring of warfarin dose with INR on a routine basis

DISPOSITION • Mortality can be reduced to 10% by rapid and effective treatment. • Mortality from recurrent pulmonary emboli is 8% with effective treatment and 30% in patients with untreated pulmonary emboli.

PEARLS & CONSIDERATIONS COMMENTS • In hemodynamically stable patients with pulmonary embolism, initial treatment with once-daily SC administration of the synthetic antithrombotic agent

fondaparinux without monitoring has been reported to be at least as safe and as effective as adjusted-dose IV unfractionated heparin. Several other trials have also demonstrated that fixed-dose low molecular weight heparin is as effective and safe as dose-adjusted IV unfractionated heparin for the initial treatment of nonmassive PE. • The duration of oral anticoagulant treatment is 6 mo in patients with reversible risk factors and indefinitely in patients with persistence of risk factors that caused the initial PE. • For the diagnosis of PE, Wells et al have developed the following clinical prediction rules to determine the probability of PE, assigning a score to each finding: 1. Clinical signs/symptoms of DVT (minimum of leg swelling and pain with palpation of the deep veins of the legs (score  3.0) 2. No alternate diagnosis likely or more likely than PE (score  3.0) 3. Heart rate 100/min (score  1.5) 4. Immobilization or surgery in last 4 wk (score  1.5) 5. Previous history of DVT or PE (score = 1.5) 6. Hemoptysis (score  1.0) 7. Cancer actively treated within last 6 months (score  1.0) • Probability of PE is high if total score is 6, moderate if 2-6, and low if  2

SUGGESTED READINGS Agnelli G et al: Extended oral anticoagulant therapy after a first episode of pulmonary embolism, Ann Intern Med 139:19, 2003. Fedullo PF, Tapson VF: The evaluation of suspected pulmonary embolism, N Engl J Med 349:1247, 2003. Quinlan DJ et al: Low-molecular weight heparin compared with IV unfractionated heparin for treatment of pulmonary embolism, Ann Intern Med 140:175, 2004. The Matisse Investigators: Subcutaneous fondaparinux versus IV unfractionated heparin in the initial treatment of pulmonary embolism, N Engl J Med 349:1695, 2003. Wells PS et al: Use of a clinical model for safe management of patients with suspected PE, Ann Intern Med 129:997, 1998. AUTHOR: FRED F. FERRI, M.D.

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Pulmonary Hypertension BASIC INFORMATION

SYNONYMS Primary pulmonary hypertension (PPH) Secondary pulmonary hypertension ICD-9CM CODES 416.0 Primary pulmonary hypertension 416.8 Secondary pulmonary hypertension

EPIDEMIOLOGY & DEMOGRAPHICS • Primary pulmonary hypertension (PPH) is rare, occurring in 2 cases per 1 million people per year, with an overall prevalence estimated at 1,300 per million. • PPH is more common in women than men (1.7:1). • Secondary pulmonary hypertension is more common than PPH. • Secondary pulmonary hypertension is the common pathophysiologic mechanism leading to cor pulmonale in patients with underlying pulmonary disease (e.g., COPD, pulmonary embolism).

PHYSICAL FINDINGS & CLINICAL PRESENTATION Primary pulmonary hypertension: • PPH is insidious and may go undetected for years. • Exertional dyspnea is the most common presenting symptom (60%). • Fatigue and weakness. • Syncope. • Chest pain. • Loud P2 component of the second heart sound. • Right-sided S4. • Jugular venous distention. • Abdominal distention/ascites. • Prominent parasternal (RV) impulse. • Holosystolic tricuspid regurgitation murmur heard best along the left fourth parasternal line that increases in intensity with inspiration. • Peripheral edema. Secondary pulmonary hypertension: • Similar to PPH but depends on the underlying cause (e.g., left-sided CHF, mitral stenosis, COPD).

DIAGNOSIS • The normal pulmonary arterial systolic pressure ranges from 18 to 30 mm Hg and the diastolic pressure ranges from 4 to 12 mm Hg. • PH is a hemodynamic diagnosis involving two stages: detection of elevated pressure in the pulmonary arteries, and characterization of this abnormality to determine its etiology by ruling out secondary causes. • Right-heart catheterization must be performed in all patients suspected of having PH to establish the diagnosis and document pulmonary hemodynamics.

• Primary pulmonary hypertension is a diagnosis of exclusion; all secondary causes as mentioned under “Etiology” must be excluded.

DIFFERENTIAL DIAGNOSIS The differential diagnosis is as listed under “Etiology.”

WORKUP • Screening for the presence of PH using Doppler echocardiography is warranted in individuals with a known predisposing genetic mutation or firstdegree relative with idiopathic PPH, scleroderma, congenital heart disease with left-to-right shunt, or portal hypertension undergoing evaluation for orthotopic liver transplantation. • The workup of a patient suspected of having PPH includes a detailed evaluation of the heart and lungs. Blood tests, chest x-ray, pulmonary function tests, CT scan of the chest, radionuclide studies of the heart and lungs, echocardiogram, electrocardiograms, pulmonary angiogram, and right- and left-heart catheterization are all required to exclude secondary causes of pulmonary hypertension. • Once the diagnosis has been made, functional assessment should be undergone to determine disease prognosis and potential treatment options. • The degree of functional impairment as assessed by the WHO classification system, and the 6-minute walk test is a useful way to monitor disease progression and assess response to treatment.

LABORATORY TESTS • CBC is usually normal in PPH but may show secondary polycythemia. • ABGs show low PO2 and oxygen saturation. • PFT is done to exclude obstructive or restrictive lung disease. • Overnight oximetry and sleep study to rule out sleep apnea/hypopnea. • ECG may show evidence of both right atrial enlargement (tall P wave 2.5 mV in leads II, III, aVF) and right ventricular enlargement (right axis deviation 100 and R wave  S wave in lead V1). • Other blood tests: ANA titer to screen for underlying connective tissue disease, HIV serology, liver function tests, and antiphospholipid antibodies. • Assessment of exercise capacity is a key part of the evaluation of PH in characterizing the disease and determining prognosis and treatment options. The 6-minute walk test and cardiopulmonary exercise testing with gas exchange measurements are the most commonly used methods of assessment.

DISEASES AND DISORDERS

Pulmonary hypertension (PH) is abnormally elevated pressure in the arterial side of the pulmonary circulation, usually defined as mean pulmonary pressure 25 mm Hg at rest or greater than 30 mmHg with exercise. Sustained elevation in pulmonary arterial pressure due to increased pulmonary venous pressure, hypoxic pulmonary vasoconstriction, or increased flow is often referred to as secondary pulmonary hypertension.

• The etiology of PPH is unknown. Most cases are sporadic, but there is a 6% to 12% familial incidence. • PPH is associated with several known risk factors: portal hypertension and liver cirrhosis, appetite-suppressant drugs (fenfluramine), and HIV disease. • Several genetic abnormalities have been associated with the familial form of PPH, many of which are mutations in the genes that code for members of the TGF-_ family of receptors (BMPRII, ALK-1) on chromosome 2q33. • Familial PPH is an autosomal-dominant disease with variable penetrance, affecting only about 10% to 20% of carriers. • Several factors have been identified that play a role in the pathogenesis of PPH, including a genetic predisposition, endothelial cell dysfunction, abnormalities in vasomotor control, thrombotic obliteration of the vascular lumen, and vascular remodeling through cell proliferation and matrix production. An emerging theory involves abnormal membrane potassium channels modulating calcium kinetics. • Secondary pulmonary hypertension is primarily caused by underlying pulmonary and cardiac conditions including: 1. Pulmonary thromboembolic disease 2. Chronic obstructive pulmonary disease (COPD) 3. Interstitial lung disease 4. Obstructive sleep disorder 5. Neuromuscular diseases causing hypoventilation (e.g., ALS) 6. Collagen-vascular disease (e.g., SLE, CREST, systemic sclerosis) 7. Pulmonary venous disease 8. Left ventricular failure resulting from hypertension, CAD, aortic stenosis, and cardiomyopathy 9. Valvular heart disease (e.g., mitral stenosis, mitral regurgitation) 10. Congenital heart disease with leftto-right shunting (e.g., ASD)

Section I

DEFINITION

ETIOLOGY

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Pulmonary Hypertension IMAGING STUDIES • Chest x-ray shows enlargement of the main and hilar pulmonary arteries with rapid tapering of the distal vessels (Fig. 1-34). Right ventricular enlargement may be evident on lateral films. • Lung perfusion scan (V/Q scan) aids in excluding chronic pulmonary embolism. • Transthoracic Doppler echocardiogram including M-mode, 2 D, pulse, continuous and color Doppler assesses ventricular function, excludes significant valvular pathology, and visualizes abnormal shunting of blood between heart chambers if present. It also provides an estimate of pulmonary artery systolic pressure that has been shown by most studies to correlate well (0.57 to 0.93) with pressures measured by right-heart catheterization. • Pulmonary angiogram is done in patients with suspicious V/Q scans. • Cardiac catheterization is performed to directly measure pulmonary artery pressures and to detect any shunting of blood.

quality of life, hemodynamics, and long-term survival in patients with WHO class III or IV function. • Inhaled aerosolized prostacyclin, iloprost, 2.5 or 5.0 µg taken 6 to 9 times per day improves exercise capacity, NYHA class, and clinical deterioration in patients with primary pulmonary hypertension and selected forms of nonprimary pulmonary hypertension. • The endothelin-receptor antagonist bosentan taken orally at a dose of 80160 mg twice daily has been approved for the treatment of PPH and scleroderma pulmonary hypertension showing improvement in clinical class and exercise capacity. Newer selective and nonselective endothelin receptor antagonists are undergoing clinical trials.

• Combination therapy using inhaled iloprost taken 1 hour before oral sildenafil 12.5 or 50 mg causes pulmonary vasodilations and, pending future trials, may be considered as treatment for pulmonary hypertension. • Lung transplantation and heart-lung transplantation are other options in end-stage class IV patients. • Lung transplant recipients with PPH had survival rates of 73% at 1 yr, 55% at 3 yr, and 45% at 5 yr.

DISPOSITION • The 6-min walk test is predictive of survival in patients with idiopathic PPH. Desaturation 10% during the test increases mortality risk 2.9 times over a median follow-up of 26 mo.

TREATMENT NONPHARMACOLOGIC THERAPY

A

• Oxygen therapy to improve alveolar oxygen flow in both primary and secondary pulmonary hypertension • Avoidance of vigorous exercise • Chest physiotherapy

ACUTE GENERAL Rx • PPH 1. Diuretics (e.g., furosemide 40-80 mg qd) improve dyspnea and peripheral edema. 2. Digoxin 0.25 mg qd has been used in patients with PPH. 3. Vasodilator treatment is usually done with hemodynamic monitoring and includes IV adenosine, prostacyclin, or nitric oxide. • Secondary pulmonary hypertension treatment is aimed at the underlying cause (see specific disease in text for treatment).

B

CHRONIC Rx • Chronic anticoagulation with warfarin is recommended to prevent thromboses and has been shown to prolong life in patients with PPH. • Calcium channel blockers may alleviate pulmonary vasoconstriction and prolong life in about 20% of patients with PPH. • Continuous infusion of epoprostenol, or prostacyclin, a short-acting vasodilator and inhibitor of platelet aggregation, improves exercise capacity,

FIGURE 1-34 Progressive pulmonary arterial hypertension. This patient initially presented with a relatively normal chest radiograph (A). However, several years later (B), there is increasing heart size as well as marked dilation of the main pulmonary artery (MPA) and right pulmonary artery (RPA). Rapid tapering of the arteries as they proceed peripherally is suggestive of pulmonary hypertension and is sometimes referred to as pruning. (From Mettler FA [ed]: Primary care radiology, Philadelphia, 2000, WB Saunders.)

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Pulmonary Hypertension

If the diagnosis of PPH is suspected, a consultation with a pulmonary specialist is recommended. Secondary causes of pulmonary hypertension may require consultations with rheumatology, neurology, and cardiology.

PEARLS & CONSIDERATIONS COMMENTS • The exertional dyspnea of PH is typically described by patients as being relentlessly progressive over several months to a year, often out of proportion to, or in the absence of, underlying heart or lung disease.

SUGGESTED READINGS Barst RJ et al: Diagnosis and differential assessment of pulmonary arterial hypertension, J Am Coll Cardiol 43:40S, 2004. Chatterjee K, De Marco T, Alpert JS: Pulmonary hypertension: hemodynamic diagnosis and management, Arch Intern Med 162:1925, 2002. Ghofrani HA et al: Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension, Ann Intern Med 136:515, 2002. Krowka MJ: Pulmonary hypertension: diagnosis and therapeutics, Mayo Clin Proc 75:625, 2000. McLaughlin VV et al: Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines, Chest 1126:78S, 2004. Nauser T, Stites S: Diagnosis and treatment of pulmonary hypertension, Am Fam Physician 63:1789, 2001. Olschewski H et al: Inhaled iloprost for severe pulmonary hypertension, N Engl J Med 347:322, 2002. Rubin LJ et al: Bosentan therapy for pulmonary arterial hypertension, N Engl J Med 346:896, 2002. AUTHORS: JASON IANNUCCILLI, M.D., and PETER PETROPOULOS, M.D.

DISEASES AND DISORDERS

REFERRAL

• Chest x-ray may reveal evidence of interstitial fluid within the lungs in cases of secondary pulmonary hypertension. PPH is not associated with infiltrates on CXR. • Factors contributing to pulmonary arterial hypertension are: 1. Alveolar hypoxia 2. Acidosis 3. Thromboemboli occluding arterial blood vessels (e.g., pulmonary embolism) 4. Scarring or destruction of alveolar walls (e.g., COPD, infiltrative disease) 5. Primary thickening of arterial walls as occurs in PPH • RVSP as estimated by echocardiography is not a very good indicator of the presence of PH, because RVSP increases with age and BMI. Athletically conditioned men also have a higher resting RVSP, and thus these measurements can be misleading. • Abrupt development of pulmonary edema during acute vasodilator testing suggests pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis and is a contraindication to chronic vasodilator treatment.

Section I

• The actual 6-min walk distance on chronic epoprostenol treatment is more predictive of survival than the change in 6-min walk distance before and after treatment. • WHO Class II and III patients with PPH have a mean survival of 3.5 yr. • WHO Class IV patients have a mean survival of 6 mo. • Logistic regression equations have been reported to predict survival or death within 1, 2, or 3 yr after diagnosis in patients with PPH.

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Renal Cell Adenocarcinoma BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Renal cell adenocarcinoma (RCA) is a primary adenocarcinoma originating in the renal parenchyma from the malignant transformation of proximal renal tubular epithelial cells.

• Transitional cell carcinomas of the renal pelvis (8% of all renal cancers) • Wilms’ tumor • Other rare primary renal carcinomas and sarcomas • Renal cysts • All causes of hematuria (see Section II) • Retroperitoneal tumors

STAGING See Table 1-13.

SYNONYMS Hypernephroma Clear cell carcinoma of the kidney Grawitz tumor

WORKUP • Laboratory tests and imaging studies

ICD-9CM CODES 189.0 Adenocarcinoma of kidney 189.1 (Renal pelvis)

Lung Bone Regional nodes Main renal vein Perirenal fat Adrenal (ipsilateral) Vena cava Brain Adjacent organs (colon, pancreas) Kidney (contralateral)

50% 30% 15% 15% 10% 10% 10% 10%

to to to to to to to to

60% 40% 30% 20% 20% 15% 15% 15% 10% 2%

LABORATORY TESTS

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE: Approximately 1:10,000 persons/yr (3% of all adult malignancies) AGE: Peak in age 50 to 70 yr SEX: Male:female ratio of 2:1

PHYSICAL FINDINGS & CLINICAL PRESENTATION Presenting findings in RCA Hematuria Elevated erythrocyte sedimentation rate Abdominal mass Anemia Flank pain Hypertension Weight loss Fever Hepatic dysfunction Classic triad (hematuria, abdominal mass, flank pain) Hypercalcemia Erythrocytosis Varicocele

COMMON SITES OF METASTASES

patients: 50% to 60% 50% 25% 20% 35% 20% 30% 5% 10%

to to to to to to to to

60% 45% 40% 40% 40% 35% 15% 15%

5% 3% 3% 2%

to to to to

10% 6% 4% 3%

• CBC: anemia or erythrocytosis • Elevated sedimentation rate • Nonmetastatic hepatic dysfunction with elevated alkaline phosphatase, prolonged prothrombin time, and hypoalbuminemia • Hypercalcemia (secondary to parathyroid related protein) • Other: elevated ferritin, elevated insulin and glucagon levels, elevated alpha-fetoprotein, and elevated betahuman chorionic gonadotropin

IMAGING STUDIES • Intravenous pyelography (IVP) • Renal ultrasound • Abdominal CT scan with contrast (Fig. 1-35) • MRI • Renal arteriogram

TREATMENT • Surgery Surgical nephrectomy is the only effective management for stages I, II, and some stage III tumors. Various forms of partial nephrectomy may be available for patients with bilateral cancers or with a solitary kidney. The role of nephrectomy in patients with metastatic renal cell carcinoma is controversial and should probably be reserved for patients who have a solitary metastasis amenable to surgical resection. • Angioinfarction (for palliation) • Radiotherapy (for palliation) • Chemotherapy (only 5% response rate)

ETIOLOGY Hereditary forms • Familial renal carcinoma • Renal carcinoma associated with von Hippel-Lindau disease • Hereditary papillary renal cell carcinoma Risk factors • Cigarette smoking • Obesity • Use of diuretics • Phenacetin-containing analgesics • Asbestos exposure • Gasoline and other petroleum products • Lead • Cadmium • Thorotrast • Role of the VHL gene located on chromosome 3

FIGURE 1-35 Large renal cell carcinoma. Large mass (M) containing areas of high enhancement, low enhancement, and necrosis. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

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Renal Cell Adenocarcinoma SUGGESTED READINGS

Prognosis of surgically treated patients TNM stage 5-year survival (%) I 95 II 88 III (renal vein or vena cava) 50 to 60 III (nodal involvement) 15 to 25 IV 5 to 20

Curti BD: Renal cell carcinoma, JAMA 292:97, 2004. Flanigan RC et al: Nephrectomy followed by interferon alfa-2b compared with interferon alfa-2b alone for metastatic renal-cell cancer, N Engl J Med 345:1655, 2002. Jennings SB, Linehan WM: Renal, perirenal, and ureteral neoplasms. In Gillenwater JY et al: Adult and Pediatric Urology, ed 3, St Louis, 1996, Mosby. Yang CJ: A randomized trial of Bevacizumab, an anti-vascular endothelial growth factor antibody, for metastatic renal cancer, N Engl J Med 349:427, 2003.

REFERRAL To urologist

AUTHOR: TOM J. WACHTEL, M.D.

TABLE 1-13

Comparison of Conventional and TNM Staging Classification of RCC

Robson Stage

T

I: Tumor confined by capsule

IIIb: Lymphatic involvement

T1 (tumor 2.5 cm or less) T2 (tumor 2.5 cm, limited to kidney) T3a (tumor invades adrenal gland or perinephric fat but not beyond Gerota’s fascia) T3b (renal vein or caval involvement below diaphragm) T3c (caval involvement above diaphragm) T1-4

IIIc: Combination of IIIa and IIIb IVa: Spread to contiguous organs except ipsilateral adrenal IVb: Distant metastases

T3, 4 T4 (tumor extends beyond Gerota’s fascia) T1-4

II: Tumor extension to perirenal fat or ipsilateral adrenal but confined by Gerota’s fascia IIIa: Renal vein or inferior vena caval involvement

N

M

N0 (nodes negative)

M0 (no distant metastases)

N1 (single lymph node 2 cm or less) N2 (single node between 2 and 5 cm, or multiple nodes 5 cm) N3 (single or multiple nodes 5 cm)

M1 (distant metastases)

DISEASES AND DISORDERS

PROGNOSIS

Section I

• Hormonal therapy (high-dose progesterone may achieve a 15% to 20% response rate) • Immunotherapy (interleukin-2 may achieve a 15% to 30% response rate; alpha, beta, and gamma interferons are somewhat less effective; for example, interferon alfa-2b increased postnephrectomy median survival by 30% in one recent trial) • Antivascular endothelial growth factor antibody Bevacizumab slows disease progression in metastatic renal cancer.

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Renal Failure, Acute BASIC INFORMATION DEFINITION Acute renal failure (ARF) is the rapid impairment in renal function resulting in retention of products in the blood that are normally excreted by the kidneys.

SYNONYMS ARF ICD-9CM CODES 584.9 Acute renal failure, unspecified

EPIDEMIOLOGY & DEMOGRAPHICS • ARF requiring dialysis develops in 5/100,000 persons annually. • 10% of ICU patients develop ARF. • 40% of hospital ARF is iatrogenic. • The most common cause of ARF in hospitalized patients is intrinsic renal failure caused by acute tubular necrosis (ATN). • Acute renal failure occurs in 20% of patients with moderate sepsis and over 50% of patients with septic shock and positive blood cultures.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • The physical examination should focus on volume status. The physical findings noted below vary with the duration and rapidity of onset of renal failure • Peripheral edema • Skin pallor, ecchymoses • Oliguria (however, patients can have nonoliguric renal failure), anuria • Delirium, lethargy, myoclonus, seizures

TABLE 1-14

• Back pain, fasciculations, muscle cramps • Tachypnea, tachycardia • Weakness, anorexia, generalized malaise, nausea

ETIOLOGY • Prerenal: inadequate perfusion caused by hypovolemia, CHF, cirrhosis, sepsis. Sixty percent of community-acquired cases of ARF are due to prerenal conditions. • Postrenal: outlet obstruction from prostatic enlargement, ureteral obstruction (stones), bilateral renal vein occlusion. Postrenal causes account for 5% to 15% of community-acquired ARF. • Intrinsic renal: glomerulonephritis, acute tubular necrosis, drug toxicity, contrast nephropathy. • Causes of acute renal failure are described in Section II.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Refer to “Etiology.”

WORKUP A thorough review of the patient’s history is necessary to identify contributing factors (e.g., nephrotoxin exposure, hypertension, diabetes mellitus). Laboratory evaluation to quantify degree of abnormality; radiographic studies to exclude prerenal and postrenal factors. Categorization of renal failure into oliguric (urinary output 400 ml/day) or nonoliguric is important. Anuria is common in obstructive uropathy and acute cortical necrosis.

LABORATORY TESTS • Elevated serum creatinine: the rate of rise of creatinine is approximately 1 mg/dl/day in complete renal failure. • Elevated BUN: BUN/creatinine ratio is 20:1 in prerenal azotemia, postrenal azotemia, and acute glomerulonephritis; it is 20:1 in acute interstitial nephritis and acute tubular necrosis (Table 1-14). • Electrolytes (potassium, phosphate) are elevated; bicarbonate level and calcium are decreased. • CBC may reveal anemia because of decreased erythropoietin production, hemoconcentration, or hemolysis. • Urinalysis may reveal the presence of hematuria (GN), proteinuria (nephrotic syndrome), casts (e.g., granular casts in ATN, RBC casts in acute GN, WBC casts in acute interstitial nephritis), eosinophiluria (acute interstitial nephritis). • Urinary sodium and urinary creatinine should also be obtained to calculate the fractional excretion of sodium (FENa) (FENa  Urine sodium/plasma sodium  Plasma creatinine/urine creatinine  100). The fractional excretion of sodium is 1 in prerenal failure, 1 in intrinsic renal failure in patients with urine output 400 ml/day. • Urinary osmolarity is 250 to 300 mOsm/kg in ATN, 400 mOsm/kg in postrenal azotemia, and 500 mOsm/kg in prerenal azotemia and acute glomerulonephritis (Table 1-15). • Additional useful studies are blood cultures for patients suspected of sepsis, LFTs, immunoglobulins, and protein electrophoresis in patients

Serum and Radiographic Abnormalities in Renal Failure Prerenal

Postrenal (Acute)

Intrinsic Renal (Acute)

Intrinsic Renal (Chronic)

BUN

↑10:1  Cr

↑ 20-40/d

↑ 20-40/d

Serum creatinine

N/moderate ↑

↑ 2-4/d

↑ 2-4/d

Serum potassium

N/moderate ↑

↑ varies with urinary volume

Serum phosphorus

N/moderate ↑

Moderate ↑ ↑↑ with rhabdomyolysis

Serum calcium

N

N/↓ with PO43 retention

↑↑ (particularly when patient is oliguric) ↑↑↑ with rhabdomyolysis ↑ Poor correlation with duration of renal disease ↓ (poor correlation with duration of renal failure)

Stable, ↑ varies with protein intake Stable ↑ (production equals excretion) Normal until end stage, unless tubular dysfunction (type 4 RTA)

Renal size By ultrasound FENa*

N/↑ 1

↑ and dilated calyces 1 → 1

N/↑ 1

Becomes significantly elevated when serum creatinine level surpasses 3 mg/dl Usually ↓ ↓ and with ↑ echogenicity 1

From Kiss B: Renal failure. In Ferri FF (ed): Practical guide to the care of the medical patient, ed 6, St Louis, 2004, Mosby. ↑, Increase; ↓, decrease; ↑↑, large increase, Cr, creatinine; N, normal; Na, sodium; P, plasma; RTA, renal tubular acidosis; U, urine. *FENa  UNa/PNaUCr/Pcr  100.

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Renal Failure, Acute • Anterograde and/or retrograde pyelogram can be used for ruling out obstruction; useful in patients at high risk of obstruction.

TREATMENT

• Stop all nephrotoxic medications • Dietary modification to supply adequate calories while minimizing accumulation of toxins; appropriate control of fluid balance. Physicians should recommend a nutrition program with an energy prescription of 120 to 150 kJ/kg per day and restriction of potassium (60 mEq/day), sodium (90 mEq/day), and phosphorus (800 mg/day). Ideal protein supplementation ranges from 0.6 to 1.4 g/kg depending on whether dialysis is required • Daily weight • Modifications of dosage of renally excreted drugs

IMAGING STUDIES • Chest x-ray is useful to evaluate for CHF and for pulmonary renal syndromes (Goodpasture’s syndrome, Wegener’s granulomatosis). • Ultrasound of kidneys is used to evaluate for kidney size (useful to distinguish ARF from CRF), to evaluate for the presence of obstruction, and to evaluate renal vascular status (with Doppler evaluation).

TABLE 1-15

ACUTE GENERAL Rx Treatment is variable with etiology of ARF: • Prerenal: IV volume expansion in hypovolemic patients • Intrinsic renal: discontinuation of any potential toxins and treatment of condition causing the renal failure • Postrenal: removal of obstruction

• Monitoring of renal function and electrolytes. • Prevention of further insults to the kidneys with proper hydration, especially before contrast studies, and avoidance of nephrotoxic agents. Hydration with sodium bicarbonate (addition of 154 ml of 1000 mEq/L sodium bicarbonate to 846 mL of 5% dextrose in water) before contrast exposure is more effective than hydration with sodium chloride for prophylaxis of contrastinduced renal failure. After appropriate clinical evaluation and measurement of blood pressure, patients should receive an initial IV bolus of 3 ml/kg/hr for 1 hr immediately before radiocontrast injection and the same fluid at a rate of 1 ml/kg/hr during the contrast exposure and for 6 hr after the procedure. • Refer to topic on chronic renal failure for indications for initiation of dialysis. Daily hemodialysis is superior to every-other-day hemodialysis in patients with acute tubular necrosis and ARF.

DISPOSITION • Prognosis is variable depending on the etiology of the renal failure, degree of renal failure, multiorgan involvement, and patient’s age. • Renal function recovery (ability to discontinue dialysis) varies from 50% to 75% in survivors of ARF.

Urinary Abnormalities in Renal Failure Prerenal

Postrenal (Acute)

Urinary volume

↓

Urinary creatinine Osmolarity Degree of proteinuria

↑ (U/P Cr 40) ↑ (400 mOsm/kg) Minimum

Urinary sediment

Negative, or occasional hyaline cast

Absent-to-wide fluctuation ↓ (U/P Cr 20) (350 mOsm/kg) Absent

Negative or hematuria with stones or papillary necrosis Pyuria with infectious prostatic disease

Intrinsic Renal (Acute)

Intrinsic Renal (Chronic)

Oliguric or nonoliguric

1000 ml  until end stage

↓ (U/P Cr 20) (350 mOsm/kg) Varies with cause of renal failure: Modest with ATN Nephrotic range common with acute glomerulopathies, usually 2 g/24 hr with interstitial disease* ATN: muddy brown Interstitial nephritis: lymphocytes, eosinophils (in stained preparations), and WBC casts RPGN: RBC casts Nephrosis: oval fat bodies

↓ (U/P Cr 20) (350 mOsm/kg) Varies with cause of renal disease (from 1-2 g/d to nephrotic range)

Broad casts with variable renal “residual” acute findings

From Kiss B: Renal failure. In Ferri FF (ed): Practical guide to the care of the medical patient, ed 6, St Louis, 2004, Mosby. Urinary concentration  Urinary volume ↑, Increased; ↓, decreased; ATN, acute tubular necrosis; clearance  Cr, creatinine; RBC, red blood cell; Plasma concentration RPGN, rapidly progressive glomerulonephritis; U/P, urine/plasma; WBC, white blood cell.

DISEASES AND DISORDERS

NONPHARMACOLOGIC THERAPY

CHRONIC Rx Section I

suspected of myeloma, creatinine kinase in patients with suspected rhabdomyolysis. • Renal biopsy may be indicated in patients with intrinsic renal failure when considering specific therapy; major uses of renal biopsy are differential diagnosis of nephrotic syndrome, separation of lupus vasculitis from other vasculitis and lupus membranous from idiopathic membranous, confirmation of hereditary nephropathies on the basis of the ultrastructure, diagnosis of rapidly progressing glomerulonephritis, separation of allergic interstitial nephritis from ATN, separation of primary glomerulonephritis syndromes. The biopsy may be performed percutaneously or by open method. The percutaneous approach is favored and generally yields adequate tissue in 90% of cases. Open biopsy is generally reserved for uncooperative patients, those with solitary kidney, and patients at risk for uncontrolled bleeding.

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Renal Failure, Acute • Overall mortality rate in ARF is nearly 50%, varying from 60% in patients with ATN to 35% in patients with prerenal or postrenal ARF. • The combination of acute renal failure and sepsis is associated with a 70% mortality rate.

REFERRAL • Nephrology consultation is recommended in renal failure. • General indications for initiation of dialysis are: 1. Florid symptoms of uremia (encephalopathy, pericarditis) 2. Severe volume overload

3. Severe acid-base imbalance 4. Significant derangement in electrolyte concentrations (e.g., hyperkalemia, hyponatremia) • Surgical consult may be necessary in patients with obstruction.

PEARLS & CONSIDERATIONS COMMENTS • It is important for physicians to recognize the growing list of medications that can result in ARF.

SUGGESTED READINGS Albright RC: Acute renal failure: a practical update, Mayo Clin Proc 76:67, 2001. Kellum JA, Decker JM: Use of dopamine in acute renal failure: a meta analysis, Crit Care Med 29:1526, 2001. Klassen PS et al: Association between pulse pressure and mortality in patients undergoing maintenance hemodialysis, JAMA 287: 1548, 2002. Merten GJ et al: Prevention of contrastinduced nephropathy with sodium bicarbonate, JAMA 291:2328, 2004. Nally JV: Acute renal failure in hospitalized patients, Cleve Clin J Med 69:569, 2002. Schiffel H et al: Daily hemodialysis and the outcome of acute renal failure, N Engl J Med 346:305, 2002. Schrier RW, Wang W: Acute renal failure and sepsis, N Engl J Med 351:159, 2004. AUTHOR: FRED F. FERRI, M.D.

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Renal Failure, Chronic BASIC INFORMATION

SYNONYMS CRF End-stage renal disease ICD-9CM CODES 585 Chronic renal failure

EPIDEMIOLOGY & DEMOGRAPHICS • The number of patients with ESRD is increasing at the rate of 7% to 9%/yr in the U.S. Each year 2/10,000 persons develop end-stage CRF. • In the U.S., 250,000/yr receive dialysis treatment for ESRD.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • • • • •

Skin pallor, ecchymoses Edema Hypertension Emotional lability and depression The clinical presentation varies with the degree of renal failure and its underlying etiology. Common symptoms are generalized fatigue, nausea, anorexia, pruritus, insomnia, taste disturbances

ETIOLOGY • Diabetes (37%), hypertension (30%), chronic glomerulonephritis (12%) • Polycystic kidney disease • Tubular interstitial nephritis (e.g., drug hypersensitivity, analgesic nephropathy), obstructive nephropathies (e.g., nephrolithiasis, prostatic disease) • Vascular diseases (renal artery stenosis, hypertensive nephrosclerosis)

DIAGNOSIS • CRF is primarily distinguished from ARF by the duration (progression over several months). • Sonographic evaluation of the kidneys reveals smaller kidneys with increased echogenicity in CRF.

WORKUP • Laboratory evaluation and imaging studies should be aimed at identifying reversible causes of acute decrements in GFR (e.g., volume depletion, urinary tract obstruction, CHF) superimposed on chronic renal disease

LABORATORY TESTS • Elevated BUN, creatinine, creatinine clearance • Urinalysis: may reveal proteinuria, RBC casts • Serum chemistry: elevated BUN and creatinine, hyperkalemia, hyperuricemia, hypocalcemia, hyperphosphatemia, hyperglycemia, decreased bicarbonate • Measure urinary protein excretion. The finding of a ratio of protein to creatinine of 1000 mg/g suggests the presence of glomerular disease • Special studies: serum and urine immunoelectrophoresis (in suspected multiple myeloma), ANA (in suspected SLE)

IMAGING STUDIES Ultrasound of kidneys to measure kidney size and to rule out obstruction

TREATMENT NONPHARMACOLOGIC THERAPY • Provide adequate nutrition and calories (147 to 168 kJ/kg/day in energy intake, chiefly from carbohydrate and polyunsaturated fats). Referral to a dietician for nutritional therapy for patients with GFR 50 ml/1.73 m2 is recommended and is now a covered service by Medicare. • Restrict sodium (approximately 100 mmol/day), potassium (60 mmol/ day), and phosphate (800 mg/day). • Adjust drug doses to correct for prolonged half-lives. • Restrict fluid if significant edema is present. • Protein restriction (0.8 g/kg/day) may slow deterioration of renal function; however, recent studies have not confirmed this benefit. There is insufficient evidence to recommend or advise against routine restriction of protein intake.

GENERAL Rx • ACE inhibitors, ARBs, and nondihydropyridine calcium channel blockers (diltiazem or verapamil) are useful in reducing proteinuria and slowing the progression of chronic renal disease, especially in hypertensive diabetic patients. A systolic blood pressure between 110 and 129 mm Hg may be beneficial in patients with urine protein excretion 1.0 g/day. Systolic BP 110 mm Hg may be associated with a higher risk for kidney disease progression. • Initiation of dialysis 1. Urgent indications: uremic pericarditis, neuropathy, neuromuscular abnormalities, CHF, hyperkalemia, seizures. 2. Judgmental indications: creatinine clearance 10 to 15 ml/min; progressive anorexia, weight loss, reversal of sleep pattern, pruritus, uncontrolled fluid gain with hypertension and signs of CHF. • Erythropoietin for anemia: 2000 to 3000 U three times a week IV/SC to maintain Hct 30% to 33%. • Diuretics for significant fluid overload (loop diuretics are preferred). • Correction of hypertension to at least 130/85 mm Hg with ACE inhibitors (avoid in patients with significant hyperkalemia), ARBs, and/or nondihydropyridene calcium channel blockers (verapamil, diltiazem) can be used in patients intolerant to ACE inhibitors or when other agents are needed to control blood pressure. • Correction of electrolyte abnormalities (e.g., calcium chloride, glucose, sodium polystyrene sulfonate for hyperkalemia), sodium bicarbonate in patients with severe metabolic acidosis. • Lipid-lowering agents in patients with dyslipidemia, target LDL cholesterol is 100 mg/dl. IATION

OF

DIALYSIS

DISEASES AND DISORDERS

Chronic renal failure (CRF) is a progressive decrease in renal function (CFR 60 ml/min for 3 mo) with subsequent accumulation of waste products in the blood, electrolyte abnormalities, and anemia.

• Resistance exercise training can preserve lean body mass, nutritional status, and muscle function in patients with moderate chronic kidney disease. • Avoid radiocontrast agents. • Smoking cessation. • Initiate hemodialysis or peritoneal dialysis (see “General Rx”). • Prompt to nephrologist is essential. Late evaluation of patients with chronic renal disease is associated with greater burden and severity of comorbid disease and shorter survival. • Kidney transplantation in selected patients.

Section I

DEFINITION

• Kidney biopsy: generally not performed in patients with small kidneys or with advanced disease • The glomerular filtration rate is the best overall indicator of kidney function. It can be estimated using prediction equations that take into account the serum creatinine level and some or all of specific variables (body size, age, sex, race). GFR calculators are available on the National Kidney Foundation Web site (http://www. kidney.org/kls/professionals/gfr_ calculator.cfm)

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Renal Failure, Chronic • Control of renal osteodystrophy with calcium supplementation and vitamin D. Starting dose of calcium carbonate is 0.5 g with each meal, increased until the serum phosphorus concentration is normalized (most patients require 5 to 10 g/day). Calcitriol 0.125 to 0.25 g/day PO is effective in increasing serum calcium concentration. Paricalcitol, a new vitamin-D analogue has been reported as more effective than calcitriol in lessening the elevations in serum calcium and phosphorus levels. • Sevelamer (Renagel) is a useful phosphate binder to reduce serum phosphate levels.

DISPOSITION • Prognosis is influenced by comorbidity of multisystem diseases. • Kidney transplantation in selected patients improves survival. The 2-yr kidney graft survival rate for living related donor transplantations is 80%, whereas the 2-yr graft survival rate for cadaveric donor transplantation is approximately 70%.

SUGGESTED READINGS Jafar TH et al: Progression of chronic kidney disease: the role of blood pressure control, proteinuria, and angiotensin-converting enzyme inhibition, Ann Intern Med 139:244, 2003. Johnson CA et al: Clinical practice guidelines for chronic kidney disease in adults, Am Fam Physician 70:869, 2004.

Kinchen KS et al: The timing of specialist evaluation in chronic kidney disease and mortality, Ann Intern Med 137:479, 2003. Levey AS: Nondiabetic kidney disease, N Engl J Med 347:1505, 2002. Lewey AS et al: National Kidney Foundation practice guidelines for chronic kidney disease: evaluation, classification, and stratification, Ann Intern Med 139:137, 2003. Lewinsky NG: Specialist evaluation in chronic kidney disease: too little too late, Ann Intern Med 137:542, 2002. Remuzzi G et al: Chronic renal diseases: renoprotective benefits of renin-angiotensin system inhibition, Ann Intern Med 136:604, 2002. Yu HT: Progression of chronic renal failure, Arch Intern Med 163:1417, 2003. AUTHOR: FRED F. FERRI, M.D.

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Restless Legs Syndrome BASIC INFORMATION

SYNONYMS “Crazy legs” (lay term) Nocturnal myoclonus ICD-9CM CODES 333.99 Nocturnal myoclonus

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 5% to 15% DEMOGRAPHICS: Age 2 yr, with no upper limit • Age 30 to 79: 10% • Age 80 and above: 19% • Prevalence increases with age (may explain a slight female overrepresentation) • Symptoms worsen with age

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Sensory nocturnal complaints usually affecting both lower extremities:  Crawly feeling or fizzle under the skin  Itching under the skin  Pain or ache  Electric shocks  Irresistible urge to move the symptomatic leg • Other words used by patients to describe the leg symptoms: creeping, burning, searing, tugging, pulling, drawing, like water flowing, worms or bugs under the skin, restless, indescribable.

ETIOLOGY • • • •

Unknown/idiopathic Hereditary (30% to 60%) Associated with iron deficiency Associated with renal failure (endstage) • Associated with peripheral neuropathy or radiculopathy • Associated with rheumatologic conditions (rheumatoid arthritis, fibromyalgia) • Medication induced (selective serotonin reuptake inhibitors)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Periodic limb movement disorder (PLMD) (repetitive limb movements [lower extremities  upper extremities] occurring during sleep; associated with arousals from sleep and daytime sleeping; patient is typically unaware, but bed partner reports restlessness or kicking during sleep) • Peripheral neuropathy and radiculopathy • Anxiety and mood disorders • Chronic fatigue • Other sleep disorders • Neuroleptic-induced akathisia • Dyskinesias while awake • Nocturnal leg cramps with or without peripheral artery disease

• History is typically diagnostic with sensitivity and specificity both 90% • Sleep logs • Polysomnography • Ambulatory recording of leg activity • CBC, iron, TIBC

TREATMENT • Dopaminergic drugs are first line: levodopa/carbidopa (Sinemet), pergolide, ropirinole, or pramipexole • Benzodiazepines • Trazodone • Opioids • Gabapentin (Neurontin)

PROGNOSIS Tendency to worsen with age

SUGGESTED READINGS Allen RP: Restless legs syndrome: epidemiology and diagnosis, Med Behav 1:36, 1998. Earley CJ: Restless legs syndrome, N Engl J Med 348:2103, 2003. Lin SC et al: Effect of pramipexole in treatment of resistant restless legs syndrome, Mayo Clin Proc 73:497, 1998. National Heart, Lung, and Blood Institute Working Group on Restless Legs Syndrome: Restless legs syndrome: detection and management in primary care, Am Fam Physician 62:108, 2000. Phillips B et al: Epidemiology of restless legs symptoms in adults, Arch Intern Med 160:2137, 2000. AUTHOR: TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

Restless legs syndrome is a primary neurologic disorder of unknown cause that manifests with both sensory and motor symptoms usually involving the lower extremities and disrupting sleep.

WORKUP Section I

DEFINITION

• Consistent symptom relief or improvement with leg movement or walking. • Sleep disruption. • Leg movements during sleep may occur but are not the prevailing complaint (unlike the urge to move). These movements are short bursts lasting 5 sec. • Physical examination is normal.

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Rhinosinusitis, Allergic BASIC INFORMATION DEFINITION Allergic rhinitis is an inflammatory disorder involving the upper respiratory tract characterized by itching of the eyes, nose, and palate and nasal congestion. It can further be classified as seasonal if this complex of symptoms occurs at a particular time of the year or perennial if the symptoms occur year-round. Common seasonal allergens include tree, grass, weed, pollen, fungi, and molds. Perennial rhinitis is often caused by such allergens as dust mites, cockroaches, animal proteins, and fungi. ICD-9CM CODES 472.0 Rhinitis 473.9 Sinusitis

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE IN U.S.: Studies estimate the prevalence to be between 9% and 40% of the population.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients can present with any combination of the following: postnasal drip, maxillary and frontal sinus pressure, pharyngitis, cough, watery eyes, itch, vertigo, sneeze, and nasal congestion. • In patients with active rhinitis the nasal mucosa must be visualized to evaluate nasal anatomy for discharge, polyps, tumors, and intact mucosa.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS In general history can differentiate allergic rhinitis from the following: infectious rhinitis, vasomotor rhinitis (idiopathic), occupational rhinitis, and rhinitis medicamentosa.

TREATMENT NONPHARMACOLOGIC THERAPY Preventive measures include allergen avoidance and environmental measures. Identifying allergens may be difficult but

in most cases can be identified by history alone. Use of bed covers impermeable to dust mite allergens has not been shown to alleviate rhinitis. Measures that have been successful in previous research include maintaining the humidity at no greater than 50% and hot water washing of bedding once per week. HEPA filters may reduce animal and dust allergens, but do not control symptoms.

INTRANSAL Rx Intranasal corticosteroid therapy is considered first line therapy for allergic rhinitis because of its limited side effect profile and significantly greater relief of nasal symptoms, sneezing, itch, and postnasal drip than oral antihistamines. Azelastine, a topical intranasal secondgeneration antihistamine, is also efficacious in relief of the total allergic symptom profile including rhinorrhea, sneezing, postnasal drip, itchy eyes/ears/throat, and nasal congestion, but is believed to be less effective than intranasal corticosteroids. In several randomized trials monotherapy with azelastine was as effective as dual therapy of azelastine plus loratadine or fexofenadine. Intranasal decongestant therapy prepared with a topical -adrenergic agonist (e.g., oxymetazoline) reduces nasal congestion but should be avoided due to risk of nasal hyperreactivity and rebound swelling. Any usage should be limited to 3 days.

ORAL Rx • First-generation oral antihistamines should be avoided due to their anticholinergic and sedating effects. They also have a negative impact on cognitive function. The second-generation oral antihistamines (loratadine, desloratadine, fexofenadine) are less sedating than the first-generation medications and are effective in relief of sneezing, rhinorrhea, and itch. The second-generation certerzidine may be more effective in relieving the symptoms of sneezing, rhinorrhea, and itch but is associated with more sedation than other second-generation oral antihistamines. In general the secondgeneration medications are minimally effective against nasal congestion.

• Short-term (i.e., 3 to 4 days) over-thecounter therapy for nasal congestion is commonly advised with pseudoephedrine, which increases pulse pressure and systolic blood pressure and should be avoided in persons with coronary artery disease and hypertension. Pseudoephedrine also tightens the urinary bladder outlet and can worsen lower urinary tract symptoms in men with benign prostatic hypertrophy. An additive effect on the lower urinary tract is seen with the combination of decongestant and antihistamine.

REFERRAL Patients with symptoms not amenable to medical treatment should be evaluated by allergist/immunologist for allergen identification and possibly immunotherapy. An ENT specialist should evaluate patients with suspected or visualized structural abnormality when anterior rhinoscopy is required for possible biopsy or surgery.

PEARLS & CONSIDERATIONS COMMENTS • Nasal inflammation can lead to obstruction of the osteomeatal complex, predisposing patients to as many as 30% and 80% of acute and chronic bacterial infections of the sinuses, respectively. • In the elderly, systemic treatments should be reserved for patients who fail intranasal therapy. • Patients who have a history of upper respiratory tract symptoms at the same time of the year probably have allergic rhinosinusitis rather than infection.

SUGGESTED READINGS Berger WE: Weighing the pros and cons of a wide array of therapies: how to get your patient’s allergic rhinitis under control, J Resp Dis 26:150-161, 2005. Dykewicz MS: Rhinitis and sinusitis, J Allergy Clin Immuno 111:S520-S529, 2003. Kaszuba SM, Baroody FM, DeTineo M: Superiority of intranasal corticosteroids compared with an oral antihistamine in the asneeded treatment of seasonal allergic rhinitis, Arch Int Med 161:2581-2592, 2001. AUTHOR: MICHAEL P. GERARDO, D.O., M.P.H.

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Rotator Cuff Syndrome BASIC INFORMATION

SYNONYMS Impingement syndrome Painful arc syndrome Internal derangement of the subacromial joint Supraspinatus syndrome ICD-9CM CODES 726.10 Rotator cuff syndrome 727.61 Rotator cuff rupture

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: 5% to 10% of the general population PREDOMINANT AGE: Uncommon under 20 yr of age PREDOMINANT SEX: More common in males than females

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Pain, often at night • Rotator cuff tenderness • Referred pain down deltoid, especially with abduction between 70 and 120 degrees (“the painful arc”) (Fig. 1-36)

ETIOLOGY

• Rest to avoid overhead activity • Ice or heat for comfort • Carefully supervised program of stretching and strengthening • Medication: NSAIDs, subacromial corticosteroid injection (once or twice at 2 wk intervals)

• • • •

Microtrauma from repetitive use Abnormally shaped acromion Shoulder instability Worsening of process by the overhead throwing motion

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • •

Shoulder instability Degenerative arthritis Cervical radiculopathy Avascular necrosis Suprascapular nerve entrapment

WORKUP • In chronic tendinitis, clinical findings similar to those seen in partial rupture • Even with complete rupture, may have full, active range of motion in shoulder

IMAGING STUDIES • Plain radiography • Ultrasonography may be useful but only in diagnosing moderately large tears

TREATMENT ACUTE GENERAL Rx

DISPOSITION • All forms are likely to respond to nonsurgical management. • Even many complete rotator cuff tears have minimal pain and little loss of function.

REFERRAL For orthopedic consultation in cases that fail to respond to medical management or in which rotator cuff tear is suspected

PEARLS & CONSIDERATIONS COMMENTS • There is considerable disagreement regarding the likelihood of recovery once a significant rotator cuff rupture has developed. • Indications for surgery vary among surgeons. • Injection is contraindicated in the presence of local infection.

SUGGESTED READINGS Biberthaler P et al: Microcirculation associated with degenerative rotator cuff lesions, J Bone Joint Surg 85A:475, 2003. Gorski JM, Schwartz LH: Shoulder impingement presenting as neck pain, J Bone Joint Surg 85:635, 2003. Green A: Chronic massive rotator cuff tears: evaluation and management, J Am Acad Orthop Surg 11:321, 2003. Tashjian RZ et al: The effect of comorbidity on self-assessed function in patients with chronic rotator cuff tears, J Bone Joint Surg 86A:355, 2004. Teefey SA et al: Detection and quantification of rotator cuff tears, J Bone Joint Surg 86A:708, 2004. Wendelboe AM et al: Associations between body mass index and surgery for rotator cuff tendinitis, J Bone Joint Surg 86A:743, 2004. FIGURE 1-36 Rotator cuff lesions are often accompanied by painful impingement of the upwardly subluxating humerus onto the acromion. Evidence for this as a cause of pain is elicited by impingement tests, for example, by forced, passive, internal rotation and abduction of the shoulder, as shown here. (From Klippel J, Dieppe P, Ferri F [eds]: Primary care rheumatology, London, 1999, Mosby.)

AUTHOR: LONNIE R. MERCIER, M.D.

DISEASES AND DISORDERS

Rotator cuff syndrome refers to a spectrum of afflictions involving the tendons of the rotator cuff (primarily the supraspinatus), ranging from simple strains and tendinitis to complete rupture with cuff-tear arthropathy.

• MRI to evaluate full- or partial-thickness tears, chronic tendinitis, and other causes of shoulder pain • Since MRI, arthrography is rarely used

Section I

DEFINITION

• Weakness in abduction or forward flexion • Increased pain with overhead activities • Atrophy in long-standing cases of complete tear • Positive “drop-arm” test (weakness of abduction against downward pressure at 90°)

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Scabies BASIC INFORMATION

ETIOLOGY

ACUTE GENERAL Rx

DEFINITION

Human scabies is caused by the mite Sarcoptes scabiei, var. hominis (Fig. 1-37).

Scabies is a contagious disease caused by the mite Sarcoptes scabiei.

DIAGNOSIS

• Following a warm bath or shower, Lindane (Kwell, Scabene) lotion should be applied to all skin surfaces below the neck (can be applied to the face if area is infested); it should be washed off 8-12 hr after application. Repeat application 1 wk later is usually sufficient to eradicate infestation. • Pruritus generally abates 24-48 hr after treatment, but it can last up to 2 wk; oral antihistamines are effective in decreasing postscabietic pruritus. • Topical corticosteroid creams may hasten the resolution of secondary eczematous dermatitis. • If the patient is a resident of an extended care facility, it is important to educate the patients, staff, family, and frequent visitors about scabies and the need to have full cooperation in treatment. Scabicide should be applied to all patients, staff, and frequent visitors, whether symptomatic or not; symptomatic family members of staff and visitors should also receive treatment. • Permethrin 5% cream (Elimite) is also effective with usually one treatment; it should be massaged into the skin from head to soles of feet; remove 8-14 hr later by washing. If living mites are present after 14 days, treat again. • A single dose (150-200 mg/kg in 6-mg tablets) of ivermectin, an antihelmintic agent, is as effective as topical lindane for the treatment of scabies. It is the best treatment for generalized crusted scabies.

ICD-9CM CODES 133.0 Scabies

EPIDEMIOLOGY & DEMOGRAPHICS • Scabies is generally acquired by sleeping with or in the bedding of infested individuals. • It is generally associated with poor living conditions and is also common in hospitals and nursing homes.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Primary lesions are caused when the female mite burrows within the stratum corneum, laying eggs within the tract she leaves behind; burrows (linear or serpiginous tracts) end with a minute papule or vesicle. • Primary lesions are most commonly found in the web spaces of the hands, wrists, buttocks, scrotum, penis, breasts, axillae, and knees. • Secondary lesions result from scratching or infection. • Intense pruritus, especially nocturnal, is common; it is caused by an acquired sensitivity to the mite or fecal pellets and is usually noted 1 to 4 wk after the primary infestation. • Examination of the skin may reveal burrows, tiny vesicles, excoriations, inflammatory papules. • Widespread and crusted lesions (Norwegian or crusted scabies) may be seen in elderly and immunocompromised patients.

DIFFERENTIAL DIAGNOSIS • • • • • • • • •

Pediculosis Atopic dermatitis Flea bites Seborrheic dermatitis Dermatitis herpetiformis Contact dermatitis Nummular eczema Syphilis Other insect infestation

WORKUP Diagnosis is made on the clinical presentation and on the demonstration of mites, eggs, or mite feces.

LABORATORY TESTS • Microscopic demonstration of the organism, feces, or eggs: a drop of mineral oil may be placed over the suspected lesion before removal; the scrapings are transferred directly to a glass slide; a drop of potassium hydroxide is added and a cover slip is applied. • Skin biopsy is rarely necessary to make the diagnosis.

TREATMENT NONPHARMACOLOGIC THERAPY Clothing, underwear, and towels used in the 48 hr before treatment must be laundered.

DISPOSITION Refractory cases usually are seen with immunocompromised hosts or patients with underlying skin diseases.

PEARLS & CONSIDERATIONS COMMENTS • Sexual partners should be notified and treated.

SUGGESTED READINGS Fawcett RS: Ivermectin use in scabies, Am Fam Physician 68:1089, 2003. Flinders DC, DeSchweinitz P: Pediculosis and scabies, Am Fam Physician 341:8, 2004. AUTHOR: FRED F. FERRI, M.D.

FIGURE 1-37 Scabies organism in a wet mount preparation. (From Mandell GL: Mandell, Douglas, and Bennett’s principles and practice of infectious diseases, ed 5, New York, 2000, Churchill Livingstone.)

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Seizure Disorders BASIC INFORMATION

SYNONYMS Epilepsy Convulsive disorders ICD-9CM CODES 345.1 Generalized convulsive epilepsy 345.4 Partial epilepsy, with impairment of consciousness 345.5 Partial epilepsy, without impairment of consciousness

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 100 cases/ 100,000 persons/yr PREVALENCE (IN U.S.): Approximately 6.5 cases/1000 persons for all types of epilepsy PREDOMINANT SEX: Males slightly higher than females GENETICS: Genetic predisposition exists for the idiopathic generalized epilepsies

PHYSICAL FINDINGS & CLINICAL PRESENTATION The hallmark for a seizure is typically its unpredictable occurrence as a spell. 1. Generalized tonic-clonic seizure (described as grand mal seizure in outdated texts) • Sequence of motor events during the seizure typically includes widespread tonic muscle contraction evolving to clonic jerking. • Typically associated with postictal confusion lasting up to several hours. • May be associated with tongue, cheek, or lip biting or urinary incontinence. • Generally normal neurologic examination. Focal deficits may be found in patients with an underlying lesion causing the seizures.

Presyncope Psychogenic phenomena • See Seizure in Section II.

ETIOLOGY

ACUTE GENERAL Rx



LABORATORY TESTS • • • • • •

Glucose, bun, creatinine, electrolytes CBC ALT, alkaline phosphatase Calcium, phosphorus, magnesium Oxygen saturation When appropriate alcohol level and toxicology screen • Lumbar puncture as indicated by history and physical examination • EEG: most valuable diagnostic tool for identifying seizure type and predicting the likelihood of recurrence

IMAGING STUDIES • MRI with contrast: modality of choice because of its high sensitivity for stroke, tumor, abscess, atrophy, and vascular malformations • CT scan without contrast if hemorrhage is suspected

TREATMENT NONPHARMACOLOGIC THERAPY Avoid sleep deprivation or environmental precipitants (e.g., photosensitive epilepsy).

• Seizures are a symptom of an underlying abnormality affecting the CNS, not a disease. • Etiology of seizures can be divided into idiopathic, symptomatic, or cryptogenic causes. • With idiopathic generalized tonicclonic seizures, there is a postulated inherited basis for the disorder. They can continue over the patient’s lifetime. • Symptomatic generalized tonic-clonic seizures and most partial seizures result from an underlying cause such as metabolic or toxic abnormalities (including medications), stroke, vascular malformations, CNS infection, brain tumor, and trauma. • Cryptogenic seizures are those without a clear underlying cause (rare onset in the elderly).

• Single seizures lasting 5 min generally require no acute pharmacologic intervention. • Status epilepticus: 30 min of continuous seizure activity or sequential seizures without full recovery of consciousness between seizures.  Maintain/protect airway.  Give oxygen.  Monitor/maintain vital signs.  Gain IV access.  Lorazepam 0.1 mg/kg IV at 2 mg/min.  If ineffective, give phosphenytoin 20 mg IV at 150 mg/min or phenytoin at 50 mg/min.  If still ineffective, use phenobarbital 20 mg/kg IV at 100 mg/min.  Thiamine and hypertonic glucose, both IV, are usually administered. • See Table 1-16.

DIAGNOSIS

ACUTE Rx

• Differential diagnosis of generalized seizures  Syncope especially when the event was unwitnessed  Psychogenic events • Differential diagnosis of partial seizures  Migraine  TIA

• Generalized tonic-clonic seizures  Sodium valproate and phenytoin are appropriate first line therapeutic agents.  Newer agents such as lamotrigine and topiramate may be better tolerated.  For each patient, anticonvulsant choice is influenced by factors such as effectiveness, cost, adverse effects,

DISEASES AND DISORDERS

Generalized tonic-clonic seizures • Generalized tonic-clonic seizures are marked by paroxysmal hypersynchronous neuronal activity involving both cerebral hemispheres resulting in loss of consciousness with tonic muscle contraction followed by rhythmic clonic contractions. The seizure may start focally in one region or hemisphere of the brain with subsequent secondary generalization. Partial seizures • In partial seizures, the onset of abnormal electrical activity originates in a focal region or lobe of the brain. Clinical manifestations may involve sensory, motor, autonomic, or psychic symptoms. Consciousness may be preserved (simple partial seizures) or impaired (complex partial seizures).



Rarely postictal neurologic deficits may be seen in the absence of specific brain lesions; they resolve in less than 48 hours. • Postictal fever usually resolves in 24 to 48 hours if there is no comorbid infection. • Aspiration pneumonitis is more common in the elderly as a complication. 2. Partial seizure • Clinical presentation is varied and depends on the site of origin of the abnormal electrical discharges. • Symptoms of simple partial seizures can include focal motor or sensory symptoms; language disturbance; olfactory, visual, or auditory hallucinations; visceral sensations, or fear or panic. • With complex partial seizures, there is a loss or reduction of awareness. This may be preceded by an aura. There may be associated automatisms or alterations in behavior. • There may be a “march” or progression of symptoms over seconds to minutes as the ictal focus spreads along the cortex. • Neurologic examination ranges from normal to focal neurologic deficits, depending on underlying cause.

Section I

DEFINITION

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Seizure Disorders ease of administration, and type of epilepsy syndrome if present.  A single seizure with an identifiable and easily correctable provoking factor (e.g., hyponatremia) does not warrant long-term use of anticonvulsants. • Partial seizures  Carbamazepine or phenytoin are common first line therapeutic agents.  Sodium valproate may also be effective.  Newer agents such as lamotrigine or oxcarbazepine may be better tolerated, but they are more expensive.

TABLE 1-16

DISPOSITION • Varies with underlying etiology. • Approximately 70% of patients are controlled with medication.

REFERRAL If uncertain about diagnosis or patient fails to respond to appropriate medication, refer to a neurologist or epilepsy specialist for further evaluation. In addition, some types of partial seizures, particularly temporal lobe epilepsy, are amenable to surgical resection.

Drug of Choice in Treatment of Epilepsy

Seizure Type

Drug of Choice

Generalized tonic-clonic, simple, and complex partial

Phenytoin, valproic acid Carbamazepine, phenobarbital Levetiracetam, zonisamide Ethosuximide Valproic acid Phenytoin, gabapentin

Absence Temporal epilepsy

Newer Drugs Generalized tonic-clonic, simple, partial, and complex partial

Lamotrigine Tiagabine Topiramate Zonisamide Oxcarbazepine Levetiracetam Gabapentin

From Ferri F (ed): Ferri’s Clinical Advisor: Instant Diagnosis and Treatment, Philadelphia, 2005, Mosby.

PEARLS & CONSIDERATIONS CAUTION EEG is normal in as many as 50% of patients; thus diagnosis is primarily by history. Usually, a single seizure is not treated with chronic anticonvulsants.

COMMENTS • Patient education information can be obtained from the Epilepsy Foundation of America, 4351 Garden City Drive, Landover, MD 20785; phone: (800) EFA-1000.

SUGGESTED READINGS Browne TR, Holmes GL: Epilepsy, N Engl J Med 344:1145, 2001. Chabolla DR: Characteristics of the epilepsies, Mayo Clin Proc 77:981, 2002. Torres MR, Ahern GL, Labiner DM: Seizures and epilepsy: an approach to diagnosis and management, JCOM 12:103-115, 2005. Wiebe S et al: A randomized controlled trial of surgery for temporal-lobe epilepsy, N Engl J Med 345:311, 2001. AUTHORS: JOHN E. CROOM, M.D., PH.D., with revisions by TOM J. WACHTEL, M.D.

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Sexual Dysfunction

DEFINITION

DIFFERENTIAL DIAGNOSIS

Sexual dysfunction implies persistent or recurrent difficulty involving the social, psychological, cognitive, hormonal, or physical functional aspects of sexual response.

• Hormonal:  Low testosterone  Diabetes mellitus  Thyroid disorders • Psychological:  Interpersonal conflict  History of sexual abuse  Depression • Physical nongonadal:  Osteoarthritis  Pelvic fracture  Cardiac disorders  Urinary incontinence  Postoperative (ostomy, mastectomy, hysterectomy) • Physical gonadal:  Vaginal atrophy  Testicular atrophy  Scar tissue • Medication induced:  Antidepressants  Antihypertensives

SYNONYMS Impotence Frigidity ICD-9CM CODES V417.0 Sexual function problem 607.84 Impotence 625.0 Dyspareunia 602.71 Inhibited sexual desire

EPIDEMIOLOGY & DEMOGRAPHICS Normal sexual function extends throughout the life span though there appears to be a linear trend toward reduced frequency of sexual encounters as people age.

PHYSICAL FINDINGS & CLINICAL PRESENTATION MALE CLINICAL PRESENTATION • Libido dysfunction • Erectile dysfunction • Ejaculatory dysfunction • Interpersonal conflict MALE PHYSICAL PRESENTATION • Neuropathy  Impaired response to Valsalva maneuver  Absent bulbocavernous or cremasteric reflexes • Peyronie’s disease • Hypogonadism FEMALE CLINICAL PRESENTATION • Libido dysfunction • Sexual arousal disorder • Orgasmic disorder • Sexual pain disorder • Interpersonal conflict FEMAL PHYSICAL PRESENTATION • Gynecologic tumors • Interstitial cystitis • Retroverted uterus • Vaginal atrophy • Vulvar or vaginal infection

ETIOLOGY • • • • • • •

Testosterone deficiency Hypogonadism Urinary incontinence Chronic pain Interpersonal conflict Males: vascular insufficiency Females: dyspareunia and vaginal dryness

WORKUP • Sexual history with emphasis on interpersonal relations • Physical examination with emphasis on musculoskeletal function, gonadal atrophy, and cardiovascular status • Medication review

LABORATORY TESTS • • • • •

Total and bioavailable testosterone Blood sugar Thyroid stimulating hormone Luteinizing hormone Prolactin

IMAGING STUDIES Penile to brachial pressure index

TREATMENT NONPHARMACOLOGIC THERAPY • Psychological counseling • Vacuum tumescent device (penile and clitoral devices FDA approved) • Penile implant • Pelvic relaxation exercises

ACUTE GENERAL Rx MALE • Testosterone replacement therapy • Phosphodiesterase inhibitors (sildenafil, vardenafil, tadalafil) • Yohimbine • Intracavernous injection (papaverine, prostaglandin E1, pentolaine)

FEMALE • Estrogen replacement therapy • Testosterone supplementation • Water soluble lubricants

DISPOSITION Patients benefit from regular follow-up and reassurance.

REFERRAL Individual and couple psychotherapy and counseling

PEARLS & CONSIDERATIONS COMMENTS • Normal sexual function is a meaningful part of a person’s function throughout his or her life span. • Resolving the sexual dysfunction of one partner may create new interpersonal conflict. • Normal sexual relations are not always between male and female spouses, and the patient’s sexual partner is not always the obvious person.

PREVENTION Physical exercise and screening

SUGGESTED READINGS Gott M, Hinchliff S: Barriers to seeking treatment for sexual problems in primary care: a qualitative study with older people, Fam Pract 20:690-695, 2003. Hazzard WR: Principles of Geriatric Medicine and Gerontology, New York, 2002, McGrawHill Professional, pp 1312-1323. MayoClinic.com: Intimacy and aging: tips for sexual health and happiness, www.mayoclinic.com AUTHOR: JOHN A. STOUKIDES, M.D., R.PH.

DISEASES AND DISORDERS

DIAGNOSIS

Section I

BASIC INFORMATION

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Sick Sinus Syndrome BASIC INFORMATION

PHYSICAL FINDINGS & CLINICAL PRESENTATION

DEFINITION

• Lightheadedness, dizziness, syncope, palpitation • Arterial embolization (e.g., stroke) associated with atrial fibrillation • Physical examination may be normal or reveal abnormalities (e.g., heart murmurs or gallop sounds) associated with the underlying heart disease

Sick sinus syndrome is a group of cardiac rhythm disturbances characterized by abnormalities of the sinus node including (1) sinus bradycardia, (2) sinus arrest or exit block, (3) combinations of sinoatrial or atrioventricular conduction defects, and (4) supraventricular tachyarrhythmias. These abnormalities may coexist in a single patient so that a patient may have episodes of bradycardia and episodes of tachycardia.

SYNONYMS Bradycardia-tachycardia syndrome ICD-9CM CODES 427.81 Sick sinus syndrome

ETIOLOGY • Fibrosis or fatty infiltration involving the sinus node, atrioventricular node, the His bundle, or its branches • In addition, inflammatory or degenerative changes of the nerves and ganglia surrounding the sinus nodes and other sclerodegenerative changes may be found

WORKUP • ECG • Ambulatory cardiac rhythm monitoring • 24-hour ambulatory ECG (Holter) (Fig. 1-38) • Event recorder • Electrophysiologic testing including sinus nodal recovery time and sino-atrial conduction time

TREATMENT • Permanent pacemaker placement if symptoms are present • The drug treatment of the tachycardia (e.g., with digitalis or calcium channel blockers) may worsen or bring out the bradycardia and become the reason for pacemaker requirement

REFERRAL

EPIDEMIOLOGY & DEMOGRAPHICS

DIAGNOSIS

To cardiologist

• Typically associated with ischemic heart disease but may occur in the presence of a normal heart

DIFFERENTIAL DIAGNOSIS

SUGGESTED READING

• • • • • •

Zipes DP, Olgin JE: Sick sinus syndrome. In Braunwald E (ed): Heart Disease: A Textbook of Cardiovascular Medicine, ed 6, Philadelphia, 2001, WB Saunders.

Bradycardia: atrioventricular block Tachycardia: atrial fibrillation Atrial flutter Paroxysmal atrial tachycardia Sinus tachycardia Syncope (see “Syncope” in Section I)

AUTHOR: TOM J. WACHTEL, M.D.

FIGURE 1-38 Brady-tachy (sick sinus) syndrome. This rhythm strip shows a narrow-complex tachycardia (probably atrial flutter) followed by a sinus pause, an AV junctional escape beat (J), and then sinus rhythm. (From Goldberger AL: Clinical electrocardiography, ed 5, St Louis, 1994, Mosby.)

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Sjögren’s Syndrome BASIC INFORMATION

SYNONYMS SS Sicca syndrome ICD-9CM CODES 710.2 Sjögren’s syndrome

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE/PREVALENCE: 1 case/2500 persons; secondary SS is just as common and can affect up to one third of SLE patients and nearly 20% of RA patients. PREDOMINANT AGE: Peak incidence is in the sixth decade. PREDOMINANT SEX: Female  male

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Dry mouth with dry lips (cheilosis), erythema of tongue (Fig. 1-39), and other mucosal surfaces, carious teeth • Dry eyes (conjunctival injection, decreased luster, and irregularity of the corneal light reflex) • Possible salivary gland enlargement and dysfunction with subsequent difficulty in chewing and swallowing food and in speaking without frequent water intake

DIAGNOSIS DIFFERENTIAL DIAGNOSIS

LABORATORY TESTS

ETIOLOGY Autoimmune disorder

• Medication-related dryness (e.g., anticholinergics) • Age-related exocrine gland dysfunction • Mouth breathing • Anxiety • Other: sarcoidosis, primary salivary hypofunction, radiation injury, amyloidosis

WORKUP Workup involves the demonstration of the following criteria for diagnosis of primary and secondary Sjögren’s syndrome: PRIMARY: • Symptoms and objective signs of ocular dryness: 1. Schirmer’s test: 8 mm wetting per 5 min 2. Positive rose bengal or fluorescein staining of cornea and conjunctiva to demonstrate keratoconjunctivitis sicca • Symptoms and objective signs of dry mouth: 1. Decreased parotid flow using Lashley cups or other methods 2. Abnormal biopsy result of minor salivary gland (focus score 2 based on average of four assessable lobules)

• Positive ANA (60% of patients) with autoantibodies anti-SS A and anti-SS B may be present. • Additional laboratory abnormalities may include elevated ESR, anemia (normochromic, normocytic), abnormal liver function studies, elevated serum 2 microglobulin levels, rheumatoid factor. • A definite diagnosis SS can be made with a salivary gland biopsy.

TREATMENT NONPHARMACOLOGIC THERAPY • Adequate fluid replacement • Proper oral hygiene to reduce the incidence of caries

ACUTE GENERAL Rx • Use artificial tears frequently. • Pilocarpine 5 mg PO qid is useful to improve dryness. A cyclosporine 0.05% ophthalmic emulsion (Restasis) may also be useful for dry eyes. Recommended dose is one drop bid in both eyes. • Cevimeline (Evoxac), a cholinergic agent with muscarinic agonist activity, 30 mg PO tid is effective for the treatment of dry mouth in patients with Sjögren’s syndrome.

CHRONIC Rx Periodic dental and ophthalmology evaluations to screen for complications

PEARLS & CONSIDERATIONS COMMENTS Unusual presentations of SS may occur in association with polymyalgia rheumatica, chronic fatigue syndrome, FUO, and inflammatory myositis.

SUGGESTED READING FIGURE 1-39 “Crocodile tongue” in SS patient. (From Noble J: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

Kassan SS, Moutsopoulos HM: Clinical manifestations and early diagnosis of Sjögren syndrome, Arch Intern Med 164:1275, 2004. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Sjögren’s syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma cell infiltration and destruction of salivary and lacrimal glands with subsequent diminished lacrimal and salivary gland secretions. • Primary: dry mouth (xerostomia) and dry eyes (xerophthalmia) develop as isolated entities. • Secondary: associated with other disorders.

• Evidence of systemic autoimmune disorder: 1. Elevated titer of rheumatoid factor 1:320 2. Elevated titer of ANA 1:320 3. Presence of anti-SS A (Ro) or antiSS B (La) antibodies SECONDARY: • Characteristic signs and symptoms of SS (described in “Physical Findings”) • Clinical features sufficient to allow a diagnosis of RA, SLE, polymyositis, or scleroderma

Section I

DEFINITION

• Purpura (nonthrombocytopenic, hyperglobulinemic, vasculitic) may be present • Evidence of associated conditions (e.g., RA or other connective disease, lymphoma, hypothyroidism, COPD, trigeminal neuropathy, chronic liver disease, polymyopathy)

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Sleep Apnea, Obstructive BASIC INFORMATION DEFINITION The American Academy of Sleep Disorders defines obstructive sleep apnea as “characterized by repetitive episodes of upper airway obstruction that occur during sleep, usually associated with a reduction in blood oxygen saturation.”

SYNONYMS Sleep apnea syndrome Obstructive sleep apnea-hypopnea syndrome ICD-9CM CODE 780.53 Obstructive sleep apnea syndrome

EPIDEMIOLOGY & DEMOGRAPHICS Obstructive sleep apnea (OSA) occurs most frequently in 40- to 65-year-old men (4%) and women (2%). The prevalence is higher in obese and hypertensive individuals.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Systemic hypertension • History of snoring, witnessed apneas, and excessive daytime somnolence • Obesity with body mass index 27 kg/m2, neck circumference 43 cm (17) in men • Working memory impairment, inability to concentrate, short tempered • Examination of oropharynx may reveal erythema caused by snoring and narrowing secondary to large tonsils, pendulous uvula, excessive soft tissue, prominent tongue and retrognathia • Patient’s bed partner may report loud snoring, episodic choking sounds, disrupted sleep with repetitive arousals, thrashing movements of extremities during sleep • Decreased libido, mood swings, depression, and chronic fatigue

ETIOLOGY Narrowing of upper airway secondary to: • Obesity • Macroglossia • Tonsillar and adenoid hypertrophy • Micrognathia • Muscular weakness • Use of alcohol or sedatives at bedtime

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Excessive Daytime Somnolence Inadequate sleep time Pulmonary disease Parkinsonism Sleep-related epilepsy

Narcolepsy Hypothyroidism • Sleep Fragmentation Sleep-related asthma Sleep-related GERD Periodic limb movement disorder Parasomnias Psychophysiologic insomnia Panic disorder Narcolepsy

WORKUP • Medical history should include questions about snoring, witnessed apneas, and excessive daytime sleepiness. Additional history concerning morning headaches, alcohol intake, weight gain, and mood/personality changes also may help to implicate apnea. • Sleep apnea can be confirmed by overnight polysomnography (gold standard). Testing is performed during the patient’s habitual sleep hours and ideally includes all stages of sleep and body positions. Patients with sleep apnea have 5 apneic/hypopneic episodes per hour (termed respiratory disturbance index, or RDI) with desaturations of at least 4% by oximetry or coincidental arousals. Overnight oximetry tests can suggest the presence of sleep apnea but are not sufficient to rule out sleep apnea. • Portable monitors that measure RDI are available but they lack the EEG, EMG, and technical observations necessary to diagnose sleep apnea with reliable accuracy. The use of such devices is usually related to limited availability of polysomnography.

LABORATORY TESTS

• Elimination of the supine sleeping position • For mild obstructive sleep apnea in select patient populations (e.g., retrognathia) an oral appliance (constructed by a qualified dentist) may be useful to push the mandible forward • Uvulopalatopharyngoplasty (UPPP, both standard and laser-assisted [LAUP]) in patients with significant obstruction of retropalatal airway • Nasal septoplasty in patients with nasoseptal deformity

ACUTE GENERAL Rx • Nighttime treatment with continuous positive airway pressure (CPAP) provides immediate resolution of sleep apnea. Symptoms of excessive daytime somnolence may linger and necessitate further investigation or medical therapy • Tracheostomy: reserved for life-threatening cases that are unresponsive to other treatments • Nasal steroids in allergy or sinusitis patients

CHRONIC Rx • CPAP therapy • Weight loss

DISPOSITION • Most patients improve with weight loss and CPAP. • Overall success rate for UPPP is about 40% for snoring; likely less effective for apnea. • Weight loss over time may reduce the need for CPAP pressure or obviate its use entirely.

• TSH level is indicated in suspected hypothyroidism. • CBC (with iron studies) is indicated for detecting anemia. • Pulmonary function tests are indicated for detecting related pulmonary disorders. • ECG is indicated for detecting related heart disease (e.g., CHF and arrhythmias).

REFERRAL

IMAGING STUDIES

• In a primary care setting, patients with high risk of sleep apnea are those who meet two of the following three criteria: (1) snoring, (2) persistent daytime sleepiness or drowsiness while driving, (3) obesity or hypertension. • Some patients with sleep apnea experience nocturnal dysrhythmias (bradycardia, paroxysmal tachyarrhythmias). In cardiac patients, trials using atrial overdrive pacing have demonstrated a significant reduction in the number of episodes of sleep apnea without reduction in the total sleep time.

Radiography of soft tissues in the neck in patients with suspected anatomic abnormalities

TREATMENT NONPHARMACOLOGIC THERAPY • Weight loss in overweight patients including bariatric surgery • Avoidance of sedating medications and alcohol • Sleep hygiene training

• Sleep physician for proper study type(s) and/or complex symptoms • Surgical referral for patients unresponsive to weight loss and CPAP • Dental referral for oral devices

PEARLS & CONSIDERATIONS

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Sleep Apnea, Obstructive

American Academy of Pediatrics: Clinical practice guideline: diagnosis and management of childhood obstructive sleep apnea syndrome, Pediatrics 109:704, 2002.

Partinen M, Hublin C: Epidemiology of sleep disorders. In Kryger M, Roth T, Dement W (eds): Principles and Practice of Sleep Medicine, ed 3, Philadelphia, 2000, WB Saunders. Schroeder BM: Obstructive sleep apnea syndrome in children, Am Fam Physician 66:1338, 2002. The International Classification of Sleep Disorders Revised, Diagnostic and Coding Manual. American Academy of Sleep Medicine, 2000. AUTHOR: J.S. DURMER, M.D., PH.D.

DISEASES AND DISORDERS

SUGGESTED READINGS

Arens, R: Obstructive sleep apnea in childhood, clinical features. In Loughlin G, Carroll J, Marcus C (eds): Sleep and Breathing in Children, New York, 2000, Marcel Dekker. Chervin RD et al: Inattention, hyperactivity and symptoms of sleep-disordered breathing, Pediatrics 109:449, 2002. Flemons WW: Obstructive sleep apnea, N Engl J Med 347:498, 2002. Garrigue A et al: Benefit of atrial pacing in sleep apnea syndrome, N Engl J Med 346:404, 2002. Marzec M et al: Effects of vagal nerve stimulation on sleep-related breathing in epilepsy patients, Epilepsia 44:930, 2003.

Section I

• The use of vagal nerve stimulators (VNS) in epilepsy patients has been associated with an increase in apneas and hypopneas. VNS-related respiratory events may be reduced by altering VNS stimulation parameters or by initiating CPAP.

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Spinal Stenosis BASIC INFORMATION

DIAGNOSIS

DEFINITION

DIFFERENTIAL DIAGNOSIS

Spinal stenosis is the pathologic condition compressing or narrowing the spinal canal, nerve root canal, or intervertebral foramina.

Spinal stenosis must be differentiated from other common causes of back and leg pain; osteoarthritis of the knee or hip, osteomyelitis, epidural abscess, metastatic tumors, multiple myeloma, intermittent claudication secondary to peripheral vascular disease, neuropathy, scoliosis, herniated nucleus pulposus, spondylolisthesis, acute cauda equina syndrome, ankylosing spondylitis, Reiter’s syndrome, fibromyalgia.

SYNONYMS Central spinal stenosis Lateral spinal stenosis Spondylosis ICD-9CM CODES 724.02 Spinal stenosis lumbar, lumbosacral

WORKUP

EPIDEMIOLOGY & DEMOGRAPHICS

The workup of spinal stenosis consists of a detailed history, physical examination, and specific imaging studies.

• More common in the elderly. • More than 30,000 patients underwent surgery for spinal stenosis in 1994.

IMAGING STUDIES

CLINICAL PRESENTATION & PHYSICAL FINDINGS • Neurogenic claudication: leg, buttock, or back pain precipitated by walking and relieved by sitting. The relief by sitting is the reason that the term pseudoclaudication is used sometimes. The mechanism of relief, however, is flexion of the spine. Look for the “shopping cart sign.” Ask the patient to pay attention for symptom relief by bending forward. • Radicular leg pain • Paresthesias • Difficulty standing or lying in an erect position • Decreased lumbar extension • Normal peripheral pulses • Positive Romberg • Wide-based gait • Reduced knee and ankle reflex • Urine incontinence

ETIOLOGY • Degenerative (hypertrophy of the articular processes, disk degeneration, ligamentum flavum hypertrophy, spondylolisthesis) • Fracture/trauma • Postoperative (postlaminectomy) • Paget’s disease • Ankylosing spondylitis • Tumors • Acromegaly

• Lumbar spine film. • CT scan of the lumbosacral spine: sensitivity (75% to 85%), specificity (80%). • MRI of the lumbosacral spine: sensitivity (80% to 90%), specificity (95%). • Myelogram: sensitivity (77%), specificity (72%). Absolute stenosis is defined as the anterior-posterior (AP) diameter of the spinal canal 10 mm. Relative stenosis: 10-12 mm AP diameter. • CT and MRI can visualize both the central and lateral canals. Electromyography (EMG) and nerve conduction velocity (NCV) are additional studies that may be useful in differentiating peripheral neuropathy from lumbar spinal stenosis.

• Surgical procedures include decompressive laminectomy, arthrodesis, hemilaminectomy, and medial facetectomy.

CHRONIC Rx • Conservative therapy with NSAIDs (ibuprofen 800 mg PO tid, naproxen 500 mg PO bid) should be tried for symptomatic relief in addition to acetaminophen 1 g PO qid. • Epidural steroid injections may provide temporary relief.

DISPOSITION • Approximately 20% of patients having surgery require repeat surgery within 10 yr. Nearly one third of the patients continue to experience pain. • The natural history of spinal stenosis is one of slow progression. In some cases symptoms improve. Although not very common, cord compression with resultant bowel and bladder incontinence and paresis can occur.

REFERRAL • Patients who have spinal stenosis unresponsive to medical treatment, and patients with symptomatic radiculopathy should be referred to an orthopedic surgeon specializing in back surgery or to a neurosurgeon. • Pain clinic referrals can be made if surgery is contraindicated or if the patient does not want surgery.

PEARLS & CONSIDERATIONS

TREATMENT

COMMENTS

NONPHARMACOLOGIC THERAPY

Approximately one third of the patients with neurogenic claudication have coexisting peripheral vascular disease.

• • • • •

Physiotherapy Lumbar corsets Back exercises Abdominal muscle strengthening Aquatic exercises

ACUTE GENERAL Rx • Surgery is indicated in patients with significant compression of nerve roots as determined by MRI or CT and incapacitating symptoms limiting activities of daily living or bladder and bowel incontinence. However, because the severity of symptoms varies over time, medical treatment should usually precede surgery in the absence of nerve root compression.

SUGGESTED READINGS Fritz JM et al: Lumbar spinal stenosis: a review of current concepts in evaluation, management, and outcome measurements, Arch Phys Med Rehabil 79:700, 1998. Schonstrom N, Willen J: Imaging lumbar spinal stenosis, Radiol Clin North Am 39(1):31, 2001. Sheehan JM, Shaffrey CI, Jane JA: Degenerative lumbar stenosis: the neurosurgical perspective, Clin Orthop 384:61, 2001. Snyder DL, Doggett D, Turkelson C: Treatment of degenerative lumbar spinal stenosis, Am Fam Physician 70:517-520, 2004. AUTHORS: PETER PETROPOULOS, M.D., with minor revisions by TOM J. WACHTEL, M.D.

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Squamous Cell Carcinoma (Skin) ETIOLOGY

DEFINITION

Risk factors include UVB radiation and immunosuppression (renal transplant recipients have a threefold increased risk).

Squamous cell carcinoma (SCC) is a malignant tumor of the skin arising in the epithelium.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS

SCC Skin cancer

• • • • • • • •

ICD-9CM CODES 173.9 Skin neoplasm, site unspecified

EPIDEMIOLOGY & DEMOGRAPHICS • SCC is the second most common cutaneous malignancy, comprising 20% of all cases of nonmelanoma skin cancer. • Incidence is highest in lower latitudes (e.g., southern U.S., Australia). • Male:female ratio of 2:1. • Incidence increases with age and sun exposure. • Average age at diagnosis is 66 yr.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • SCC commonly affects scalp, neck region, back of hands, superior surface of the pinna, and the lip. • The lesion may have a scaly, erythematous macule or plaque. • Telangiectasia, central ulceration may also be present (Fig. 1-40). • Most SCC present as exophytic lesions that grow over a period of months.

Keratoacanthomas Actinic keratosis Amelanotic melanoma Basal cell carcinoma Benign tumors Healing traumatic wounds Spindle cell tumors Warts

WORKUP Diagnosis is made with full-thickness skin biopsy (incisional or excisional).

TREATMENT ACUTE GENERAL Rx • Electrodesiccation and curettage for small SCCs (2 cm in diameter), superficial tumors and lesions located in extremity and trunk. • Tumors thinner than 4 mm can be managed by simple local removal. • Lesions between 4 and 8 mm thick or those with deep dermal invasion should be excised. • Tumors penetrating the dermis can be treated with several modalities, including excision and Mohs’ surgery, radiation therapy, and chemotherapy.

DISPOSITION • Survival is related to size, location, degree of differentiation, immunologic status of the patient, depth of invasion, and presence of metastases. Risk factors for metastasis include lesions on the lip or ear, increasing lesion depth, and poor cell differentiation. • Patients whose tumors penetrate through the dermis or exceed 8 mm in thickness are at risk of tumor recurrence. • The most common metastatic locations are regional lymph nodes, liver, and lung. • Tumors on the scalp, forehead, ears, nose, and lips also carry a higher risk. • SCCs originating in the lip and pinna metastasize in 10% to 20% of cases. • Five-year survival for metastatic squamous cell carcinoma is 34%.

REFERRAL Oncology referral for metastatic SCC

PEARLS & CONSIDERATIONS COMMENTS SCC arising in areas of prior radiation, thermal injury, and areas of chronic ulcers or chronic draining sinuses are more aggressive and have a higher frequency of metastases than those originating in actinic damaged skin. AUTHOR: FRED F. FERRI, M.D.

FIGURE 1-40 Squamous cell carcinoma. Nodular hyperkeratotic lesion with central erosion. (From Noble J et al: Textbook of primary care medicine, ed 3, St Louis, 2001, Mosby.)

DISEASES AND DISORDERS

SYNONYMS

• Metastatic SCC (rare) can be treated with cryotherapy and combination of chemotherapy using 13-cis-retinoic acid and interferon -2A.

Section I

BASIC INFORMATION

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Stasis Dermatitis BASIC INFORMATION DEFINITION Stasis dermatitis refers to an inflammatory skin disease of the lower extremities, commonly seen in patients with chronic venous insufficiency (Fig. 1-41). ICD-9CM CODES 459.81 Stasis dermatitis

EPIDEMIOLOGY & DEMOGRAPHICS

• Venous insufficiency subsequently results in venous hypertension, causing skin inflammation and the aforementioned physical findings and clinical presentation.

DIAGNOSIS The diagnosis of stasis dermatitis is primarily made by a detailed history and physical examination.

DIFFERENTIAL DIAGNOSIS

• Stasis dermatitis occurs more frequently in the elderly • Rarely seen before the age of 50 yr • Estimated to occur in up to 6% to 7% of the patients 50 yr • Occurs in women more often than men, perhaps related to lower extremity venous impairment aggravated through pregnancy

• • • • • • • • • •

PHYSICAL FINDINGS & CLINICAL PRESENTATION

WORKUP

• Insidious onset • Pruritus • Chronic edema usually described as “brawny” edema as stasis dermatitis pathologically is associated with dermal fibrosis • Erythema • Scaly • Eczematous patches • Commonly located over the medial malleolus • Progressive pigment changes can occur as a result of extravasation of red blood cells and hemosiderin deposition within the cutaneous tissue. • Secondary infections can occur

ETIOLOGY • Stasis dermatitis is thought to occur as a direct result from any insult or injury of the lower extremity venous system leading to venous insufficiency including: 1. Deep vein thrombosis 2. Trauma 3. Pregnancy 4. Vein stripping 5. Vein harvesting in patients requiring coronary artery bypass grafting (CABG)

Contact dermatitis Atopic dermatitis Cellulitis Tinea dermatophyte infection Pretibial myxedema Nummular eczema Lichen simplex chronicus Xerosis Asteatotic eczema Deep vein thrombosis

The workup of a patient with stasis dermatitis is directed at excluding potential life-threatening causes (e.g., deep vein thrombosis) and complications (e.g., cellulites and sepsis).

LABORATORY TESTS Blood tests are generally not very helpful unless a secondary infection is present.

IMAGING STUDIES • X-rays, CT scans, and MRIs are generally not very helpful. • Doppler studies are indicated in any patient suspected of having a deep vein thrombosis.

TREATMENT NONPHARMACOLOGIC THERAPY • Leg elevation • Compression stocking with a gradient of at least 30-40 mm Hg • For weeping skin lesions, wet to dry dressing changes are helpful

ACUTE GENERAL Rx • In patients with acute stasis dermatitis, a compression (Unna) boot can be applied. An Unna boot consists of a roll

FIGURE 1-41 Moderate stasis dermatitis with hyperpigmentation and bilateral venous insufficiency. (Courtesy Department of Dermatology, University of North Carolina at Chapel Hill. From Goldstein BG, Goldstein AO: Practical dermatology, ed 2, St Louis, 1997, Mosby.)

of gauze that is saturated with zinc oxide ointment supported with an elastic wrap. Unna boots are most effective for ambulatory patients. Nonambulatory patients do better with compression devices (elastic or pneumatic). • Topical corticosteroid creams or ointments (e.g., triamcinolone 0.1% bid) are used frequently to help reduce inflammation and itching. • Secondary infections should be treated with appropriate antibiotics. Most secondary infections are the result of Staphylococcus or Streptococcus organisms.

CHRONIC Rx • Patients with chronic stasis dermatitis can be treated with topical emollients (e.g., white petrolatum, lanolin, Eucerin). • Topical dressings (e.g., DuoDerm) are effective in the treatment of chronic venous stasis ulcers.

DISPOSITION • The mainstay of treatment of stasis dermatitis is to control leg edema and prevent venous stasis ulcers from developing. • Chronic venous stasis ulcers may take months to heal and may require skin grafting.

REFERRAL • Dermatology referral is made if the diagnosis is unclear. • Vascular surgery referral is made for assistance in the management of chronic venous insufficiency and chronic venous stasis ulcers.

PEARLS & CONSIDERATIONS COMMENTS • Inflammatory skin changes from stasis dermatitis are thought to result from poor oxygen perfusion to the lowerextremity skin tissue. Various theories, including venous pooling, arteriovenous shunting, increased venous hydrostatic pressure affecting microcirculation, fibrin barriers preventing oxygen diffusion, and leukocyte trapping with resultant microvascular damage, have all been hypothesized as causes of stasis dermatitis. • Diuretic use should be avoided or limited to intermittent short courses.

SUGGESTED READINGS Flugman SL et al: Stasis dermatitis, www.emedicine.com. Yuwono HS: Diagnosis and treatment in the management of chronic venous insufficiency, Clin Hemorheol Microcirc 23(24):233, 2000. AUTHOR: PETER PETROPOULOS, M.D.

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Stroke BASIC INFORMATION Stroke describes acute brain injury caused by decreased blood supply or hemorrhage. Cerebrovascular accident (CVA) ICD-9CM CODES 436 Acute stroke

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • Occurs in 10 to 20/100,000 persons 65 yr of age PREVALENCE (IN U.S.): Estimated at 2 million persons PREDOMINANT SEX: Incidence is 30% higher in males PREDOMINANT AGE: 60 yr PEAK INCIDENCE: 80-84 yr

PHYSICAL FINDINGS & CLINICAL PRESENTATION Motor and/or sensory and/or cognitive deficits, depending on distribution and extent of involved vascular territory. More common manifestations include contralateral motor weakness or sensory loss, as well as language difficulties (aphasia; predominantly left-sided lesions) and visuospatial/neglect phenomena (predominantly right-sided lesions). Onset is usually sudden; however, this depends on specific etiology (Table 1-17).

ETIOLOGY • 70%-80% are caused by ischemic infarcts; 20%-30% are hemorrhagic. • 80% of ischemic infarcts are from occlusion of large or small vessels caused by atherosclerotic vascular disease (due to hypertension, hyperlipidemia, tobacco abuse), 15% are caused by cardiac embolism, 5% are from other causes, including hypercoagulable states and vasculitis.

BOX 1-4

DIAGNOSIS

• CT scan without contrast to distinguish hemorrhage from infarct (Figs. 1-42 and 1-43) • An MRI is superior to CT in identifying abnormalities in the posterior fossa and, in particular, lacunar (small vessel) infarcts. Diffusion weighted imag-

DIFFERENTIAL DIAGNOSIS • TIA (Transient ischemic attack, traditionally defined as focal neurologic deficits lasting 24 hr [usually lasting 60 min]) • Migraine • Seizure • Mass lesion

WORKUP • Thorough history and physical examination, including detailed neurologic and cardiovascular evaluation to identify vascular territory and likely etiology (Table 1-17). Infectious, toxic, and metabolic causes should be excluded because each may cause clinical deterioration of old stroke symptoms. • Cardiac: mandatory ECG, telemetry, consider serial cardiac enzymes; transthoracic and/or transesophageal echocardiography Holter monitor should be seriously considered especially in setting of suspected embolic etiology. Carotid Doppler should be performed in cases of embolic stroke to anterior or middle cerebral artery territory.

LABORATORY TESTS • • • • • • • • • •

CBC Platelet count PT (INR) PTT BUN, creatinine Lipid panel Glucose Electrolytes Urinalysis Additional tests, depending on suspected etiology (in younger patients; e.g., coagulopathies)

FIGURE 1-42 Intracerebral hemorrhage. Noncontrast CT scan demonstrates an intracerebral hemorrhage in the right occipital lobe. (From Specht N [ed]: Practical guide to diagnostic imaging, St Louis, 1998, Mosby.)

Risk Factors for Ischemic Stroke

Diabetes Hypertension Smoking Family history of premature vascular disease Hyperlipidemia Atrial fibrillation History of transient ischemic attack (TIA) History of recent myocardial infarction History of congestive heart failure (left ventricular [LV] ejection fraction, 25%) Drugs (sympathomimetics, oral contraceptive pill, cocaine) From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders.

FIGURE 1-43 Occipital lobe infarct (posterior cerebral artery territory). Note the large right occipital hypodensity with mass effect caused by infarction and subsequent edema. (From Cwinn AA, Grahovac SZ [eds]: Emergency CT scans of the head: a practical atlas, St Louis, 1998, Mosby.)

DISEASES AND DISORDERS

SYNONYMS

IMAGING STUDIES Section I

DEFINITION

• Small vessel occlusion is most often caused by lipohyalinosis precipitated by chronic hypertension. • Risk factors for ischemic stroke are described in Box 1-4.

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Stroke TABLE 1-17

Neurologic Signs Associated with Cerebrovascular Accident by Location

Artery Affected

Neurologic Signs

Internal Carotid Artery (Supplies the cerebral hemispheres and diencephalon by the ophthalmic and ipsilateral hemisphere arteries)

Occasional unilateral blindness Severe contralateral hemiplegia, hemianesthesia, and hemianopia Profound aphasia if left hemisphere involved

Middle Cerebral Artery (Supplies structures of higher cerebral processes of communication; language interpretation; perception and interpretation of space, sensation, form, and voluntary movement) Anterior Cerebral Artery (Supplies medial surfaces and upper convexities of frontal and parietal lobes and medial surface of hemisphere, which includes motor and somesthetic cortex serving the legs) Posterior Cerebral Artery (Supplies medial and inferior temporal lobes, medial occipital lobe, thalamus, posterior hypothalamus, and visual receptive area) Vertebral or Basilar Arteries (Supply the brainstem and cerebellum) Incomplete occlusion

Anterior portion of pons Complete occlusion or hemorrhage

Posterior Inferior Cerebellar Artery (Supplies the lateral and posterior portion of the medulla)

Anterior Inferior and Superior Cerebellar Arteries (Supply the cerebellum) Anterior Spinal Artery (Supplies the anterior spinal cord) Posterior Spinal Artery (Supplies the posterior spinal cord)

Alterations in communication, cognition, mobility, and sensation Homonymous hemianopia Contralateral hemiplegia or hemiparesis

Emotional lability Confusion, amnesia, personality changes Urinary incontinence Impaired mobility, with weakness greater in lower extremities than in upper Homonymous hemianopia Hemianesthesia Cortical blindness Memory deficits Drop attacks Unilateral and bilateral weakness of extremities Diplopia, homonymous hemianopia Nausea, vertigo, tinnitus, and syncope Dysphagia Dysarthria Sometimes confusion and drowsiness “Locked-in” syndrome—no movement except eyelids; sensation and consciousness preserved Coma Miotic pupils Decerebrate rigidity Respiratory and circulatory abnormalities Death Wallenberg syndrome Dysphagia, dysphonia Ipsilateral anesthesia of face and cornea for pain and temperature (touch preserved) Ipsilateral Horner syndrome Contralateral loss of pain and temperature sensation in trunk and extremities Ipsilateral decompensation of movement (cerebellar signs) Difficulty in articulation, swallowing, gross movements of limbs; nystagmus (cerebellar signs) Flaccid paralysis, below level of lesion Loss of pain, touch, temperature sensation (proprioception preserved, sensory level) Sensory loss, particularly proprioception, vibration, touch, and pressure (movement preserved)

Adapted from Seidel HM (ed): Mosby’s guide to physical examination, ed 4, St Louis, 1999, Mosby.

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Stroke

NONPHARMACOLOGIC THERAPY • To prevent pulmonary emboli, abovethe-knee elastic stockings, pneumatic boots, or SQ heparin if nonhemorrhagic etiology and patient is immobile in bed • Carotid endarterectomy (CEA) is recommended in patients with carotid territory stroke associated with 70% to 99% ipsilateral carotid stenosis, performed by an experienced surgeon who has demonstrated low morbidity and mortality • Modification of risk factors (e.g., smoking cessation, exercise, diet)

BOX 1-5

• Box 1-5 describes initial considerations for patients with stroke. • Judicious control of blood pressure; patients with chronic hypertension may extend the area of infarction if the blood pressure is lowered into the “normal” range. It is best not to lower blood pressure too aggressively in the acute setting unless it is very markedly elevated. Adequate hydration and bed rest (e.g., head of bed down in pressure dependent ischemia vs. head of bed up if patient is aspiration risk). Tight glycemic control is also recommended (e.g., sliding scale insulin). • Patients presenting 3 hr after onset of a nonhemorrhagic stroke, thrombolytic therapy in a specialized stroke center is beneficial in selected populations.

ACUTE SPECIFIC Rx • Depends on several factors, including etiology, vascular territory involved, risk factors, and elapsed time from symptom onset to arrival at hospital. • Box 1-6 describes criteria for thrombolytic therapy in patients with thromboembolic stroke (IV tPA inclusion cri-

teria include clearly defined symptom onset within 3 hr of onset of treatment, measurable deficit with NIH Stroke Scale 4, and no evidence for bleed on neuroimaging). • If atrial fibrillation and/or a cardiac mural thrombus is found on echocardiography, heparin may be considered. • If a subarachnoid or intracerebral hemorrhage is found on CT, MR angiography and/or cerebral angiography may be indicated to identify aneurysm. If no aneurysm is found and clot is expanding, neurosurgical evacuation of clot may be attempted, but outcomes are generally poor. • In select cases of patients presenting 3 hr but 6 hr, an interventional neuroradiologist or neurosurgeon may be able to offer either direct injection of a clot-busting agent (such as intraarterial tPA) or direct extraction of the clot (e.g., FDA approved Merci Retrieval System). However, this remains investigational and has yet to be well studied in the setting of a controlled trial. Intracranial angioplasty/stenting may also be a consideration.

Initial Considerations for Patients with Strokes

Initial care Stabilize the patient, secure the airway, and provide adequate oxygenation Assess level of consciousness, language, visual fields, eye movements, and pupillary movements Obtain history and perform physical examination Perform CT of head without contrast Obtain CBC with platelets and differential, electrolytes, creatinine, BUN, glucose, PT/PTT, arterial blood gas, or oxygen saturation Consider a toxicology screen Consider special coagulation studies such as antiphospholipid antibodies, factor V Leiden assay, protein C and protein S, antithrombin III, ANA, fibrinogen, RPR, homocysteine, serum protein electrophoresis Consider acute intervention with t-PA if symptoms for less than 3 hr Consider the following with admission orders Transthoracic echocardiogram (consider transesophageal echocardiogram if transthoracic echocardiogram is equivocal or there is a high suspicion of cardiogenic thromboembolism) Carotid duplex ultrasonography Telemetry Supplemental oxygen and appropriate oxygen saturation monitoring Antiplatelet therapy Fluid restriction if infarct is large, to reduce cerebral edema Close monitoring of intake and output Regular determinations of blood glucose levels to avoid hyperglycemia NPO if there are concerns about the pharyngeal reflex pending swallowing evaluation Elevate the head of the bed 20-30 degrees to reduce cerebral edema Bed rest for the first 24 hr with fall precautions, then advance as appropriate Vital signs and neurologic checks every 2 hr times four until stable Prophylaxis for DVT if immobile (elastic stockings at a minimum) Speech therapy consultation to evaluate swallowing Neurology, physical therapy, occupational therapy, nutrition, and social services consultations From Rakel RE (ed): Principles of family practice, ed 6, Philadelphia, 2002, WB Saunders. ANA, Antinuclear antibodies; BUN, blood urea nitrogen; CBC, complete blood count; CT, computed tomography; DVT, deep vein thrombosis; NPO, nothing by mouth; PT/PTT, prothrombin time/partial thromboplastin time; RPR, rapid plasma reagin; t-PA, tissue plasminogen activator.

DISEASES AND DISORDERS

TREATMENT

ACUTE GENERAL Rx

Section I

ing (DWI) is best to determine hyperacute ischemia (positive within 15-30 min of symptom onset). MRA is recommended to help identify vascular pathology (e.g., extent of intracranial atherosclerosis or vascular distribution of ischemia) • In select cases (e.g., hemorrhagic stroke), conventional angiography may identify aneurysms or other vascular malformations

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Stroke CHRONIC Rx

REFERRAL

• Antiplatelet therapy (aspirin, dipyridamole/aspirin [Aggrenox], clopidogrel [Plavix], or ticlopidine) reduces the risk of subsequent stroke. • If patient presents with first TIA/stroke and was on no prior antiplatelet agent, aspirin (325 mg vs. 81 mg each day) is usually chosen initially. If a TIA occurs while on aspirin, the patient should be switched to dipyridamole/aspirin or clopidogrel. • Warfarin is usually reserved for patients with cardioembolic stroke as well as for patients with atrial fibrillation.

• Neurology/neurosurgical referral depending on etiology and resources available; depending on time of symptom onset, transfer of patient to institution able to provide more specific acute treatment can be considered. • Vascular surgery if patient is candidate for CEA. If not a surgical candidate and endovascular treatment is available, refer to interventional neuroradiologist for carotid stenting.

DISPOSITION Prognosis depends on severity of deficits, etiology, and other concurrent medical/surgical illness. A polymodality physical medicine and rehabilitative approach is an integral part of poststroke recovery. This includes physical, occupational, and speech therapy individualized depending on deficits.

SUGGESTED READINGS American Heart Association Scientific Statement: Primary prevention of ischemic stroke: a statement for health care professionals from the stroke council of the American Heart Association, Circulation 103:167, 2001. Barnett HJM: A modern approach to posterior circulation ischemic stroke, Arch Neurol 59:359, 2002. Benevante D, Hart RG: Stroke: management of acute ischemic stroke, Am Fam Physician 59:2828, 1999. Caplan LR: Stroke treatment: promising but still struggling, JAMA 279:1304, 1998.

Diener HC et al: Aspirin and clopidogrel compared with clopidogrel alone after recent ischaemic stroke or transient ischaemic attack in high-risk patients (MATCH): randomised, double-blind, placebo-controlled trial, Lancet 364(9431):331, 2004. Endovascular versus surgical treatment in patients with carotid stenosis in the Carotid and Vertebral Artery Transluminal Angioplasty Study (CAVATAS): a randomised trial, Lancet 357(9270):1729, 2001. Green DM et al: Serum potassium level and dietary potassium intake as risk factors for stroke, Neurology 59:314, 2002. Halperin JL, Fuster V: Patent foramen ovale and recurrent stroke: another paradoxical twist, Circulation 105:2580, 2002. Meschia JF et al: Thrombolytic treatment of acute ischemic stroke, Mayo Clin Proc 77:542, 2002. Qureshi A et al: Spontaneous intracranial hemorrhage, N Engl J Med 344:1450, 2001. Sacco RL et al: High-density lipoprotein cholesterol and ischemic stroke in the elderly, JAMA 285:2729, 2001. Strauss SE et al: New evidence for stroke prevention, clinical applications and scientific review, JAMA 288:1388, 2002. AUTHOR: RICHARD S. ISAACSON, M.D.

Criteria for Tissue Plasminogen Activator (alteplase [Activase]) Use in Patients with Thromboembolic Stroke BOX 1-6

Criteria for considering t-PA as a treatment option Noncontrast CT without evidence of hemorrhage Time since onset of symptoms clearly 3 hr before t-PA administration would begin Criteria for excluding t-PA as a treatment option Historical and clinical findings Clinical presentation suggests subarachnoid hemorrhage, even if CT is normal Sudden, severe headache, often with loss of consciousness at onset Vomiting common Active internal bleeding, increased risk of bleeding, or known bleeding diathesis, including: Recent use of warfarin with a prolonged international normalized ratio (INR)—some would add current use of warfarin regardless of INR Use of heparin within 48 hr with a prolonged aPTT Platelet count 100,000/mm3 History of intracranial hemorrhage Known arteriovenous malformation or aneurysm GI or GU bleeding within the past 21 days Arterial puncture within the past 7 days Recent lumbar puncture Stroke, intracranial surgery, or head trauma within the previous 3 mo Major surgery or serious trauma within the preceding 14 days Persistent systolic blood pressure 185 mm Hg or diastolic blood pressure 110 mm Hg Seizure at stroke onset Rapidly improving neurologic signs Isolated, mild neurologic deficits Acute myocardial infarction Post–myocardial infarction pericarditis Blood glucose 50 mg/dl or 400 mg/dl CT findings Evidence of intracranial hemorrhage Hypodensity or effacement of the sulci in 1⁄3 of the territory of the middle cerebral artery From Rakel RE (ed): Principles of family practice, ed 6, Philadelphia, 2002, WB Saunders. aPTT, Activated partial thromboplastin time; CT, computed tomography; GI, gastrointestinal; GU, genitourinary; t-PA, tissue plasminogen activator.

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Subdural Hematoma

DEFINITION

Traumatic rupture of cortical bridging veins, especially where stretched by underlying cerebral atrophy.

• There is an increased risk of seizures, which should be treated appropriately if they arise. • Reverse anticoagulation where appropriate.

DIAGNOSIS

DISPOSITION

DIFFERENTIAL DIAGNOSIS

Referral to neurosurgery for possible evacuation

A subdural hematoma is bleeding into the subdural space, caused by rupture of bridging veins between the brain and venous sinuses. ICD-9CM CODES 432.1 Subdural hematoma

EPIDEMIOLOGY & DEMOGRAPHICS Nearly all cases are caused by trauma, although the trauma may be quite trivial and easily overlooked. Victims are commonly at the extremes of age. Coagulation abnormalities, especially use of anticoagulation in the elderly, is a significant risk factor.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Vague headache, often worse in morning than evening. • Some apathy, confusion, and clouding of consciousness is common, although frank coma may complicate late cases. Chronic subdural hematomas may cause a dementia picture. • Neurologic symptoms may be transient, simulating TIA. • Almost any sign of cortical dysfunction may occur, including hemiparesis, sensory deficits, or language abnormalities, depending on which part of the cortex the hematoma presses on. • New-onset seizures should raise the index of suspicion.

A

• • • • •

Epidural hematoma Subarachnoid hemorrhage Mass lesion (e.g., tumor) Ischemic stroke Intraparenchymal hemorrhage

WORKUP • CT scan is sensitive for diagnosis and should be performed in a timely fashion (Fig. 1-44). • Hematocrit, platelet count, PTT, and PT/INR should be routinely checked.

TREATMENT NONPHARMACOLOGIC THERAPY Small subdural hematomas may be left untreated and the patient observed, but if there is an underlying cause, such as anticoagulation, this should be rapidly corrected to prevent further accumulation of blood.

ACUTE THERAPY • Neurosurgical drainage of blood from subdural space via burr hole is the definitive procedure, although it is common for the hematoma to reaccumulate.

PEARLS & CONSIDERATIONS • The elderly are particularly susceptible to subdural hematomas because of cerebral atrophy, which stretches subdural veins, and because of fall risk. • Relatively minor trauma may cause a subdural hematoma. • Caution should be taken in interpreting CT findings in the subacute stage, where blood appears as isodense to brain, and therefore the distance from the cortical sulci to the skull needs to be evaluated.

SUGGESTED READINGS Chen JC, Levy ML: Causes, epidemiology, and risk factors of chronic subdural hematoma, Neurosurg Clin N Am 11(3):399, 2000. Voelker JL: Nonoperative treatment of chronic subdural hematoma, Neurosurg Clin N Am 11(3):507, 2000. AUTHOR: DANIEL MATTSON, M.D., M.SC.(MED.)

B

FIGURE 1-44 Subdural hematomas. A noncontrasted computed tomography scan of an acute subdural hematoma (A) shows a crescentic area of increased density in the right posterior parietal region between the brain and the skull (black and white arrows). An area of intraparenchymal hemorrhage (H) is also seen; a chronic subdural hematoma for a different patient is shown in (B). There is an area of decreased density in the left frontoparietal region (arrows) effacing the sulci, compressing the anterior horn of the left lateral ventricle, and shifting the midline somewhat to the right. (From Mettler FA [ed]: Primary care radiology, Philadelphia, 2000, WB Saunders.)

DISEASES AND DISORDERS

ETIOLOGY

Section I

BASIC INFORMATION

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Syncope BASIC INFORMATION DEFINITION Syncope is the temporary loss of consciousness resulting from an acute global reduction in cerebral blood flow. ICD-9CM CODES 720.2 Syncope

EPIDEMIOLOGY & DEMOGRAPHICS • Syncope accounts for 3% to 5% of emergency room visits. • 30% of the adult population will experience at least one syncopal episode during their lifetime. • Incidence of syncope is highest in elderly men and young women.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Blood pressure: if low, consider orthostatic hypotension; if unequal in both arms (difference 20 mm Hg), consider subclavian steal or dissecting aneurysm. (NOTE: Blood pressure and heart rate should be recorded in the supine and standing positions.) If there is drop in BP but no change in HR, the patient may be on a beta blocker or may have an autonomic neuropathy. • Pulse: if patient has tachycardia, bradycardia, or irregular rhythm, consider arrhythmia. • Heart: if there are murmurs present suggestive of AS or IHSS, consider syncope secondary to left ventricular outflow obstruction; if there are JVD and distal heart sounds, consider cardiac tamponade. • Carotid sinus pressure: can be diagnostic if it reproduces symptoms and other causes are excluded; a pause 3 sec or a systolic BP drop 50 mm Hg without symptoms or 30 mm Hg with symptoms when sinus pressure is applied separately on each side for 5 sec is considered abnormal. This test should be avoided in patients with carotid bruits or cerebrovascular disease; ECG monitoring, IV access, and bedside atropine should be available when carotid sinus pressure is applied.

ETIOLOGY • Neurally mediated syncope 1. Psychophysiologic (emotional upset, panic disorders, hysteria) 2. Visceral reflex (micturition, defecation, food ingestion, coughing, ventricular contraction; glossopharyngeal neuralgia) 3. Carotid sinus pressure 4. Reduction of venous return caused by Valsalva maneuver • Orthostatic hypotension 1. Hypovolemia 2. Vasodilator medications

3. Autonomic neuropathy (diabetes, amyloid, Parkinson’s disease, multisystem atrophy) 4. Pheochromocytoma 5. Carcinoid syndrome • Cardiac 1. Reduced cardiac output a. Left ventricular outflow obstruction (aortic stenosis, hypertrophic cardiomyopathy) b. Obstruction to pulmonary flow (pulmonary embolism, pulmonic stenosis, primary pulmonary hypertension) c. MI with pump failure d. Cardiac tamponade e. Mitral stenosis f. Reduction of venous return (atrial myxoma, valve thrombus) g. -blockers 2. Arrhythmias or asystole a. Extreme tachycardia (160 to 180 bpm) b. Severe bradycardia (30 to 40 bpm) c. Sick sinus syndrome d. AV block (second- or thirddegree) e. Ventricular tachycardia or fibrillation f. Long QT syndrome g. Pacemaker malfunction h. Psychotropic medications and beta blockers 3. Other causes a. Hypoxia b. Hypoglycemia c. Anemia d. Hyperventilation

DIAGNOSIS DIFFERENTIAL DIAGNOSIS 1. Seizure (see “Workup”) 2. Vertebrobasilar TIA usually manifests as diplopia, vertigo, ataxia but not loss of consciousness. Isolated syncopal episodes without accompanying neurologic symptoms are unlikely to be a TIA 3. Recreational drugs/alcohol 4. Psychologic stress

WORKUP The history is crucial to diagnosing the cause of syncope and may suggest a diagnosis that can be evaluated with directed testing: • Sudden loss of consciousness: consider cardiac arrhythmias. • Gradual loss of consciousness: consider orthostatic hypotension, vasodepressor syncope, hypoglycemia. • History of aura before loss of consciousness (LOC) or prolonged confusion (1 min), amnesia, or lethargy after LOC suggests seizure rather than syncope.

• Patient’s activity at the time of syncope: 1. Micturition, coughing, defecation: consider syncope secondary to decreased venous return. 2. Turning head or while shaving: consider carotid sinus syndrome. 3. Physical exertion in a patient with murmur: consider aortic stenosis. 4. Arm exercise: consider subclavian steal syndrome. 5. Assuming an upright position: consider orthostatic hypotension. 6. Postprandial syncope • Associated events: 1. Chest pain: consider MI, pulmonary embolism. 2. Palpitations: consider arrhythmias. 3. Incontinence (urine or fecal) and tongue biting are associated with seizure or syncope. 4. Brief, transient shaking after LOC may represent myoclonus from global cerebral hypoperfusion and not seizures. However, sustained tonic/clonic muscle action is more suggestive of seizure. 5. Focal neurologic symptoms or signs point to a neurologic event such as a seizure with residual deficits (e.g., Todd’s paralysis) or cerebral ischemic injury. 6. Psychologic stress: syncope may be vasovagal. • Review current medications, particularly antihypertensive and psychotropic drugs.

LABORATORY TESTS Routine blood tests rarely yield diagnostically useful information and should be done only if they are specifically suggested by the results of the history and physical examination. The following are commonly ordered tests. • CBC to rule out anemia, infection • Electrolytes, BUN, creatinine, magnesium, calcium to rule out electrolyte abnormalities and evaluate fluid status • Serum glucose level • Cardiac isoenzymes should be obtained if the patient gives a history of chest pain before the syncopal episode • ABGs to rule out pulmonary embolus, hyperventilation (when suspected) • Evaluate drug and alcohol levels when suspecting toxicity

IMAGING STUDIES • Echocardiogram is useful in patients with a heart murmur to rule out AS, IHSS, or atrial myxoma. • If seizure is suspected, CT scan and/or MRI of the head and EEG may be useful. • If head trauma or neurologic signs on examination, CT or MRI may be helpful.

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Syncope

PSYCHIATRIC EVALUATION • Generalized anxiety disorder, pain disorder, and major depression predispose patients to neurally mediated reactions and may result in syncope. • Alcohol and drug dependence can also lead to syncope.

NONPHARMACOLOGIC THERAPY

REFERRAL Hospital admission in elderly patients without prior history of syncope or unknown etiology of their syncope and in any patients suspected of having cardiac syncope.

• Ensure proper hydration; consider TED stockings and salt tablets. • Eliminate medications that may induce hypotension.

ACUTE GENERAL Rx

PEARLS & CONSIDERATIONS

• Varies with the underlying etiology of syncope (e.g., pacemaker in patients with syncope secondary to complete heart block) • Syncope caused by orthostatic hypotension is treated with volume replacement in patients with intravascular volume depletion. Also consider midodrine to promote venous return via adrenergic-mediated vasoconstriction and Florinef for its mineralocorticoid effects to increase intravascular volume

COMMENTS • Section III, Syncope, describes an algorithmic approach to the patient. • The etiology of syncope is identified in 50% of cases during the initial evaluation. • A thorough history and physical examination are the most productive means of establishing a diagnosis in patients with syncope.

DISPOSITION Prognosis varies with the age of the patient and the etiology of the syncope. Generally: • Benign prognosis (very low 1-yr morbidity) in patients age 70 yr and having vasovagal/psychogenic syncope or syncope of unknown cause • Poor prognosis (high mortality and morbidity) in patients with cardiac syncope • Patients with the following risk factors have a higher 1-yr mortality: abnormal

SUGGESTED READINGS Fenton AM et al: Vasovagal syncope, Ann Intern Med 133:722, 2000. Kapoor WN: Syncope, N Engl J Med 343:1856, 2000. Menozzi C et al: Mechanism of syncope in patients with heart disease and negative electrophysiologic test, Circulation 105:2741, 2002. Soteriades ES et al: Incidence and prognosis of syncope, N Engl J Med 347:878, 2002. AUTHOR: SEAN I. SAVITZ, M.D.

120 Head up

Head down

120

Heart rate (bpm)

100 80 80

40 60

Mean blood pressure (mm Hg)

• Useful to support a diagnosis of neurally mediated syncope. Patients older than age 50 should have stress testing before tilt-table testing. Positive results would preclude tilt-table testing. • Indicated in patients with recurrent episodes of unexplained syncope as well as for patients in high-risk occupations (e.g., pilots, bus drivers) (Fig. 1-45). The test is also useful for identifying patients with prominent bradycardic response who may benefit from implantation of a permanent pacemaker. • It is performed by keeping the patient in an upright posture on a tilt table with footboard support. The angle of the tilt table varies from 60 to 80 degrees. The duration of upright posture during tilt-table testing varies from 25 to 45 min. • The hallmark of neurally mediated syncope is severe hypotension associated with a paradoxical bradycardia triggered by a specific stimulus. The diagnosis of neurally mediated syncope is likely if upright tilt testing reproduces these hemodynamic changes in 15 min and causes presyncope or syncope.

ECG, history of ventricular arrhythmia, history of CHF

0

40 1

6

12

18

24

Minutes FIGURE 1-45 Head-up tilt test performed on an 18-year-old woman with a history of syncope associated with pain, preceded by a prodrome of dizziness, graying vision, and diaphoresis. A similar prodrome preceded syncope during the test. Note the precipitous, nearly simultaneous, decline of heart rate and blood pressure after an initial rise in heart rate. Vital signs returned to normal rapidly after the head was lowered. (Courtesy Robert F. Sprung, University of Utah. In Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

DISEASES AND DISORDERS

TILT-TABLE TESTING

TREATMENT

Section I

• If pulmonary embolism is suspected, ventilation-perfusion scan should be done. • If arrhythmias are suspected, a 24-hr Holter monitor and admission to a telemetry unit is appropriate. Generally, Holter monitoring is rarely useful, revealing a cause for syncope in 3% of cases. Loop recorders that can be activated after syncopal episode to retrieve information about the cardiac rhythm during the preceding 4 min add considerable diagnostic yield in patients with unexplained syncope. • Implantable cardiac monitors that function as permanent loop recorders or implantable cardioverter-defibrillators, which are placed subcutaneously in the pectoral region with the patient under local anesthesia, are useful in patients with cardiac syncope. • Electrophysiologic studies may be indicated in patients with structural heart disease and/or recurrent syncope. • ECG to rule out arrhythmias; may be diagnostic in 5% to 10% of patients.

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Tardive Dyskinesia BASIC INFORMATION DEFINITION Tardive dyskinesia (TD) is a syndrome of involuntary movements associated with the long-term use of antipsychotic medication, particularly dopamine-blocking neuroleptics. Patients usually exhibit rapid, repetitive, stereotypic movements mostly involving the oral, buccal, and lingual areas. ICD-9CM CODES 333.82 Tardive dyskinesia

EPIDEMIOLOGY & DEMOGRAPHICS • The disorder is caused by dopamineblocking neuroleptics (e.g., Haldol). The incidence is declining with the use of newer generation antipsychotics. • At least 20% of patients treated with standard neuroleptic drugs are affected with TD, and approximately 5% are expected to develop TD with each year of neuroleptic treatment. • The risk is greatest in the early years of exposure. • Higher incidence and lower remission rates are seen in older persons.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Typically appears with the reduction or withdrawal of the antipsychotics • Characterized by: 1. TD primarily involves the tongue, lips, and jaw. A combination of tongue twisting and protrusion, lip smacking and puckering, and chewing movements in a repetitive and stereotypic fashion is often observed. 2. Slow, writhing movements of the arms and legs 3. Symptoms subside when the antipsychotic is reintroduced

4. The involuntary mouth movements in TD may be voluntarily suppressed by patients. They are also suppressed by voluntary actions such as putting food in the mouth or talking.

ETIOLOGY Tardive dyskinesia results from chronic exposure to dopamine receptor blocking agents—drugs primarily used to treat psychosis. TD has not been reported with dopamine depleters (such as reserpine) and are seldom reported with atypical antipsychotic drugs such as clozapine. Some drugs for nausea (such as metoclopramide and prochlorperazine) and depression (such as amoxapine) can also cause TD.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Huntington’s chorea • Excessive treatment with L-dopa

WORKUP • Complete neuropsychiatric history (including medication history) and examination. • If presentation atypical, consider evaluation with CBC, serum electrolytes, thyroid function tests, serum ceruloplasmin, and connective tissue disease screen.

IMAGING STUDIES Brain imaging normal in TD.

TREATMENT NONPHARMACOLOGIC THERAPY None

PHARMACOLOGIC THERAPY • Treatment predicated on prevention— limiting the indications for neuroleptics and using the lowest effective dose and withdrawn when feasible. • Use atypical antipsychotics if possible. Clozapine and quetiapine have the lowest reported incidence of TD.

CHRONIC Rx • Benzodiazapines and vitamin E may be helpful but controlled trial evidence is weak. • Clozapine, olanzapine and amisulpride may be of symptomatic help, but long-term efficacy is unproven.

REFERRAL Movement disorder specialist if symptoms are severe

PEARLS & CONSIDERATIONS Only as a last resort, for persistent, disabling, and treatment-resistant TD, should neuroleptics be resumed to treat TD in the absence of active psychosis.

SUGGESTED READINGS Casey DE: Pathophysiology of antipsychotic drug-induced movement disorders, J Clin Psychiatry 65(suppl9):25, 2004. Fernandez HH, Friedman JH: Classification and treatment of tardive syndromes, Neurologist 9(1):16, 2003. McGrath JJ, Soares KV: Miscellaneous treatments for neuroleptic-induced tardive dyskinesia, Cochrane Database Syst Rev (2):CD000208, 2003. AUTHOR: MITCHELL D. FELDMAN, M.D., M.PHIL.

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Tarsal Tunnel Syndrome BASIC INFORMATION

ICD-9CM CODES 355.5 Tarsal tunnel syndrome

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Unknown PREDOMINANT SEX: Female  male

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Neuritic symptoms along the course of the posterior tibial nerve in the sole and heel • Swelling over tarsal tunnel • Possible positive Tinel’s sign

DIAGNOSIS

• NSAIDs • Immobilization for 4-6 wk with ankle orthosis or fracture cast boot • Medial heel wedge or orthotic to minimize heel eversion • Local steroid injection into tunnel (avoiding the posterior tibial nerve) if symptoms persist

DIFFERENTIAL DIAGNOSIS

REFERRAL

• • • • • •

For surgical decompression if needed. Results of surgery are mixed unless an obvious compressive lesion is found.

ETIOLOGY Space-occupying lesions (ganglia, varicosities, lipomas, synovial hypertrophy)

Plantar fasciitis Peripheral neuropathy Proximal radiculopathy Local tendinitis Peripheral vascular disease Morton’s neuroma

ELECTRICAL STUDIES Electrodiagnostic testing is often inconclusive. Delayed sensory conduction or increased motor latency may be seen.

SUGGESTED READINGS Aldridge T: Diagnosing heel pain in adults, Am Fam Physician 70:332, 2004. Gorter K et al: Variation in diagnosis and management of common foot problems by GPs, Fam Pract 18(6):569, 2001. Labib SA et al: Heel pain triad (HPT): the combination of plantar fasciitis, posterior tibial tendon dysfunction and tarsal tunnel syndrome, Foot Ankle Int 23(3):212, 2002. Mizel MS et al: Evaluation and treatment of chronic ankle pain, Instr Course Lect 53:311, 2004. Mondelli M, Morana P, Padua L: Anelectrophysiological severity scale in tarsal tunnel syndrome, Acta Neurol Scand 109:284, 2004. Pecina M: Diagnostic tests for tarsal tunnel syndrome, J Bone Joint Surg Am 84-A(9):1714, 2002. AUTHOR: LONNIE R. MERCIER, M.D.

FIGURE 1-46 Anatomy of tarsal tunnel syndrome. Transverse view of ankle. FDL, Flexor digitorum longus; FHL, flexor hallucis longus tendon; TN, tibial nerve (single contour), posterior tibial artery, veins; TP, tibialis posterior tendon. Tendons and neurovascular elements are included into individual fibrous septa that connect periosteum with the deep fascia. (From Canoso J: Rheumatology in primary care, Philadelphia, 1997, WB Saunders.)

DISEASES AND DISORDERS

Tarsal tunnel syndrome is a rare entrapment neuropathy that develops as a result of compression of the posterior tibial nerve in the tunnel formed by the flexor retinaculum behind the medial malleolus of the ankle (Fig. 1-46).

TREATMENT Section I

DEFINITION

• Possible reproduction of symptoms with sustained eversion of hindfoot or digital compression of tunnel • Sensory and motor changes unusual

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Tendinitis, Bicipital BASIC INFORMATION DEFINITION The biceps muscle has two heads: • The short head arises from the tip of the coracoid process of the scapula with the coracobrachialis. • The long head arises from the supraglenoid tubercle of the scapula and runs through the intertubercular groove between the greater and lesser tubercles of the humerus. • The two heads join in the distal arm to form one strong tendon, which inserts on the radial tuberosity on the upper end of the radius. • Tendinitis results from a repetitive stress leading to microscopic tears in the tendon that can generate an inflammatory response. • Tenosynovitis occurs as a result of a direct injury or irritation in which the tendon rubs over a bony prominence and produces inflammation of the tendon sheath. • Tendinosis refers to degenerative change in the tendon. Tendinitis, tenosynovitis, and tendinosis often coexist.

SYNONYMS Tendinitis ICD-9CM CODES

EPIDEMIOLOGY & DEMOGRAPHICS Shoulder pain is common in all age groups and in both sexes, but the incidence of bicipital tendinitis is unknown and cases are often misclassified as rotator cuff tendinitis and vice versa.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Patients with bicipital tendinitis typically describe pain in the region of the anterior shoulder, which may radiate to the elbow. • The pain is aggravated by shoulder flexion, forearm supination, or elbow flexion. The pain may be exacerbated by beginning the activity but improves as the activity continues. The pain then may return after the patient ceases the activity.

• Physical examination reveals tenderness localized over the bicipital groove. Active shoulder abduction, flexion, and internal rotation aggravate the pain. • Flexion against resistance: with the forearm in the supinated position and the elbow fully extended the patient attempts to flex the arm (forward flexion at the shoulder) against the resistance provided by the examiner. Tenderness in the bicipital groove is considered a positive test result and is indicative of bicipital tendinitis.

ETIOLOGY • The biceps tendon is susceptible to overuse injuries, especially in individuals performing repetitive lifting activities. Degenerative changes associated with aging predispose the elderly patient to injury. • Known causes of bicipital tendinitis include the following:  Poor lifting techniques  Chronic repetitive upper extremity activities  Lack of flexibility  Anatomic abnormalities • Often no specific cause can be identified.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • •

Myofascial pain Rotator cuff disease or injury Adhesive capsulitis Brachial neuritis Cervical radiculopathy Musculocutaneous neuropathy Shoulder instability Acromioclavicular injury or arthritis

WORKUP The history and physical examination are usually sufficient to make the diagnosis of bicipital tendinitis. Local anesthetic infiltration of the region of the bicipital groove may help make the diagnosis. • Electromyography or nerve conduction studies to rule out neuropathy and radiculopathy. • Ultrasound or MRI may assist to rule out other shoulder pathology (e.g., rotator cuff tear).

TREATMENT NONPHARMACOLOGIC THERAPY • Avoid activity that aggravates the condition. • Physical therapy:  If there is no shoulder instability, perform gradual stretching of biceps and anterior shoulder capsule.  Circumduction, pendulum, twohand rod swinging, and lateral and front finger wall walking may begin as symptoms abate.  Perform progressive resistance exercises as soon as symptoms subside.  Apply moist heat to help facilitate stretching and reduce pain.  Physical therapy should involve soft tissue therapy with massage.  Electrical stimulation or ultrasound may be beneficial if symptoms interfere with therapeutic exercise.

PHARMACOLOGIC THERAPY • Nonsteroidal antiinflammatory drugs. • Topical capsaicin (0.075% strength applied tid). • Injection with anesthetic (e.g., 1.5 cc of lidocaine 1% or 2%) and corticosteroid (e.g., triamcinolone 20 to 40 mg) into the biceps tendon sheath in the intertubercular groove. May be repeated on a 4-week basis. • Myofascial trigger point injections with dilute local anesthetic in the parascapular muscles may be beneficial. • Surgery may be required in refractory cases with persistent pain that has not responded to any other treatments. Surgery often involves decompression of the musculotendinous structure through tenolysis under arthroscopy or using an open surgical technique.

SUGGESTED READINGS Barr KP: Rotator cuff disease, Phys Med Rehabil Clin N Am 15(2):475-491, 2004. Mehta S, Gimbel JA, Soslowsky LJ: Etiologic and pathogenetic factors for rotator cuff tendinopathy, Clin Sports Med 22(4):791812, 2003. AUTHOR: TOM J. WACHTEL, M.D.

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Thrombophlebitis, Superficial BASIC INFORMATION Superficial thrombophlebitis is inflammatory thrombosis in subcutaneous veins.

SYNONYMS ICD-9CM CODES 451.0 Thrombophlebitis, superficial

EPIDEMIOLOGY & DEMOGRAPHICS • 20% of superficial thrombophlebitis cases are associated with occult DVT. • Catheter-related thrombophlebitis incidence is 100:100,000.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Subcutaneous vein is palpable, tender; tender cord is present with erythema and edema of the overlying skin and subcutaneous tissue. • Induration, redness, and tenderness are localized along the course of the vein. This linear appearance rather than circular appearance is useful to distinguish thrombophlebitis from other conditions (cellulitis, erythema nodosum). • There is no significant swelling of the limb (superficial thrombophlebitis generally does not produce swelling of the limb). • Low-grade fever may be present. High fever and chills are suggestive of septic phlebitis.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • •

Lymphangitis Cellulitis Erythema nodosum Panniculitis Kaposi’s sarcoma

WORKUP Laboratory evaluation to exclude infectious etiology and imaging studies to rule out DVT in suspected cases

TREATMENT NONPHARMACOLOGIC THERAPY • Warm, moist compresses • It is not necessary to restrict activity; however, if there is extensive thrombophlebitis, bed rest with the leg elevated will limit the thrombosis and improve symptoms.

ACUTE GENERAL Rx • NSAIDs to relieve symptoms • Treatment of septic thrombophlebitis with antibiotics with adequate coverage of Staphylococcus • Ligation and division of the superficial vein at the junction to avoid propagation of the clot in the deep venous system when the thrombophlebitis progresses toward the junction of the involved superficial vein with deep veins

DISPOSITION

LABORATORY TESTS

Clinical improvement within 7-10 days

CBC with differential, blood cultures, culture of IV catheter tip (when secondary to intravenous cannulation)

REFERRAL Surgical referral in selected cases (see “Acute General Rx”)

IMAGING STUDIES • Serial ultrasound or venography in patients with suspected DVT • CT scan of abdomen in patients with suspected malignancy (Trousseau’s syndrome: recurrent migratory thrombophlebitis)

PEARLS & CONSIDERATIONS COMMENTS • Patients with positive cultures should be evaluated and treated for endocarditis. • Septic thrombophlebitis is more common in IV drug addicts. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

Phlebitis

• Trauma to preexisting varices • Intravenous cannulation of veins (most common cause) • Abdominal cancer (e.g., carcinoma of pancreas) • Infection (Staphylococcus is the most common pathogen) • Hypercoagulable state • DVT

Section I

DEFINITION

ETIOLOGY

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Thrombosis, Deep Vein BASIC INFORMATION DEFINITION Deep vein thrombosis (DVT) is the development of thrombi in the deep veins of the extremities or pelvis.

SYNONYMS DVT Deep venous thrombophlebitis ICD-9CM CODES 451.1 Thrombosis of deep vessels of lower extremities 451.83 Thrombosis of deep veins of upper extremities 541.9 Deep vein thrombosis of unspecified site

EPIDEMIOLOGY & DEMOGRAPHICS • Annual incidence in urban population is 1.6 cases/1000 persons. • The risk of recurrent thromboembolism is higher among men than women.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Pain and swelling of the affected extremity • In lower extremity DVT, leg pain on dorsiflexion of the foot (Homans’ sign) • Physical examination may be unremarkable

ETIOLOGY The etiology is often multifactorial (prolonged stasis, coagulation abnormalities, vessel wall trauma). The following are risk factors for DVT: • Prolonged immobilization (3 days) • Postoperative state • Trauma to pelvis and lower extremities • High-dose estrogen therapy; conjugated equine estrogen but not esterified estrogen is associated wtih increased risk of DVT; estrogen plus progestin is associated with doubling the risk of venous thrombosis • Visceral cancer (lung, pancreas, alimentary tract, GU tract) • Age 60 yr • History of thromboembolic disease • Hematologic disorders (e.g., antithrombin III deficiency, protein C deficiency, protein S deficiency, heparin cofactor II deficiency, sticky platelet syndrome, G20210A prothrombin mutation, lupus anticoagulant, dysfibrinogenemias, anticardiolipin antibody, hyperhomocystinemia, concurrent homocystinuria, high levels of factors VIII, XI, and factor V Leiden mutation) • Obesity, CHF • Surgery, fracture, or injury involving lower leg or pelvis

• Surgery requiring 30 min of anesthesia • Gynecologic surgery (particularly gynecologic cancer surgery) • Recent travel (within 2 wk, lasting 4 hr) • Smoking and abdominal obesity • Central venous catheter or pacemaker insertion • Superficial vein thrombosis, varicose veins

trasonography can be safely omitted in such patients. • Laboratory evaluation of patients with recurrent thrombosis without obvious causes and those with a family history of thrombosis should include protein S, protein C, fibrinogen, antithrombin III level, lupus anticoagulant, anticardiolipin antibodies, factor V Leiden, factor VIII, factor IX, and plasma homocysteine levels.

DIAGNOSIS

IMAGING STUDIES

DIFFERENTIAL DIAGNOSIS • • • • • • • • • • •

Postphlebitic syndrome Superficial thrombophlebitis Ruptured Baker’s cyst Cellulitis, lymphangitis, Achilles tendinitis Hematoma Muscle or soft tissue injury, stress fracture Varicose veins, lymphedema Arterial insufficiency Abscess Claudication Venous stasis

WORKUP The clinical diagnosis of DVT is inaccurate. Pain, tenderness, swelling, or color changes are not specific for DVT. Compression ultrasonography is preferred as the initial study to diagnose DVT. An initial negative test should be repeated after 5 days (if the clinical suspicion of DVT persists) to detect propagation of any thrombosis to the proximal veins. Comprehensive ultrasonography is a more extensive test, which examines the deep veins from the inguinal ligament to the level of the malleolus. Recent literature reports indicate that it may be safe to withhold anticoagulation after negative results on comprehensive duplex ultrasonography in patients with a suspected first episode of symptomatic DVT of the leg.

LABORATORY TESTS • Laboratory tests are not specific for DVT. Baseline PT (INR), PTT, and platelet count should be obtained on all patients before starting anticoagulation. • Use of D-dimer assay by ELISA may be useful in the management of suspected DVT. The combination of a normal D-dimer study on presentation together with a normal compression venous ultrasound is useful to exclude DVT and generally eliminate the need to do repeat ultrasound at 5-7 days. Recent trials indicate that DVT can be ruled out in patients who are clinically unlikely to have DVT and who have a negative D-dimer test. Compressive ul-

• Compression ultrasonography is generally preferred as the initial study because it is noninvasive and can be repeated serially (useful to monitor suspected acute DVT); it offers good sensitivity for detecting proximal vein thrombosis (in the popliteal or femoral vein). Its disadvantages are poor visualization of deep iliac and pelvic veins and poor sensitivity in isolated or nonocclusive calf vein thrombi. • Contrast venography is the gold standard for evaluation of DVT of the lower extremity. It is, however, invasive and painful. Additional disadvantages are the increased risk of phlebitis, new thrombosis, renal failure, and hypersensitivity reaction to contrast media; it also gives poor visualization of deep femoral vein in the thigh and internal iliac vein and its tributaries. • Magnetic resonance direct thrombus imaging (MRDTI) is an accurate noninvasive test for diagnosis of DVT. Current limitations are its cost and lack of widespread availability.

TREATMENT NONPHARMACOLOGIC THERAPY • Initial bed rest for 1-4 days followed by gradual resumption of normal activity • Patient education on anticoagulant therapy and associated risks

ACUTE GENERAL Rx • Traditional treatment consists of IV unfractionated heparin for 4 to 7 days followed by warfarin therapy. Low–molecular-weight heparin enoxaparin (Lovenox) is also effective for initial management of DVT and allows outpatient treatment. Recommended dose is 1 mg/kg q12h SC and continued for a minimum of 5 days and until a therapeutic INR (2-3) has been achieved with warfarin. Once-daily fondaparinux (Arixtra), a synthetic analog of heparin, is also as effective and safe as twice daily enoxaparin in the initial treatment of patients with symptomatic

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Thrombosis, Deep Vein

•

•

CHRONIC Rx • Conventional-intensity warfarin therapy is more effective than lowintensity warfarin therapy for the long term prevention of recurrent DVT. The low-intensity warfarin regimen does not reduce the risk of clinically important bleeding. • The optimal duration of anticoagulant therapy varies with the cause of DVT and the patient’s risk factors: 1. Therapy for 3-6 mo is generally satisfactory in patients with reversible risk factors (low-risk group). 2. Anticoagulation for at least 6 mo is recommended for patients with idiopathic venous thrombosis or medical risk factors for DVT (intermediate-risk group) 3. Indefinite anticoagulation is necessary in patients with DVT associated with active cancer; long-term anticoagulation is also indicated in patients with inherited thrombophilia (e.g., deficiency of protein C or S antibody), antiphospholipid, and those with recurrent episodes of idiopathic DVT (high-risk group).

PEARLS & CONSIDERATIONS COMMENTS • When using heparin, there is a risk of heparin-induced thrombocytopenia (with unfractionated more so than with LMWH). Platelet count should be obtained initially and repeated every 3 days while on heparin. • Prophylaxis of DVT is recommended in all patients at risk (e.g., low–molecular-weight heparin [enoxaparin 30 mg SC bid] after major trauma, post surgery of hip and knee; enoxaparin 40 mg SC qd post–abdominal surgery in patients with moderate to high DVT risk; gradient elastic stockings alone or in combination with intermittent pneumatic compression [IPC] boots following neurosurgery). • Ximelagatran is an oral direct thrombin inhibitor. For prophylaxis of venous thromboembolism, ximelagatran 24 mg PO bid started the morning after total knee arthroplasty is well tolerated and at least as effective as warfarin, but it does not require coagulation monitoring or dose adjustment. • Fondaparinux (Arixtra), a synthetic analog of heparin, can also be used for prevention of DVT after hip fracture surgery, hip replacement, or knee replacement. Initial dose is 2.5 mg SC given 6 to 8 hr postoperatively and continued daily. Its bleeding risk is similar to enoxaparin; however, it is more effective in preventing DVT. • The risk of recurrent venous thromboembolism in heterozygous carriers of factor V Leiden and a first spontaneous venous thromboembolism is similar to that of noncarriers of factor V Leiden; therefore heterozygous patients should receive secondary thromboprophylaxis for a similar length of time as patients without factor V Leiden. • Approximately 20%-50% of patients with DVT develop postthrombotic syndrome characterized by leg edema, pain, venous ectasia, skin induration, and ulceration.

• Exercise following DVT is reasonable because it improves flexibility of the affected leg and does not increase symptoms in patients with postthrombotic syndrome.

SUGGESTED READINGS Baarslag et al: Prospective study of color duplex ultrasonography compared with contrast venography in patients suspected of having deep venous thrombosis of the upper extremities, Ann Intern Med 136:865, 2002. Bates SM, Ginsberg JS: Treatment of deep-vein thrombosis, N Engl J Med 351:268, 2004. Berquist D et al: Duration of prophylaxis against venous thromboembolism with enoxaparin after surgery for cancer, N Engl J Med 346:975, 2002. Eichinger S et al: D-Dimer levels and risk of recurrent venous thromboembolism, JAMA 290:1071, 2003. Francis CW et al: Ximelagatran versus warfarin for the prevention of venous thromboembolism after total knee arthroplasty, Ann Intern Med 137:648, 2002. Fraser DG et al: Diagnosis of lower-limb deep venous thrombosis: a prospective blinded study of magnetic resonance direct thrombus imaging, Ann Intern Med 136:89, 2002. Kahn SR et al: Acute effects of exercise in patients with previous DVT: impact of the post-thrombotic syndrome, Chest 123:399, 2003. Kelly et al: Plasma D-Dimers in the diagnosis of venous thromboembolism, Arch Intern Med 162:747, 2002. Kovacs MJ et al: Comparison of 10-mg and 5mg warfarin initiation nomograms together with low-molecular-weight heparin for outpatient treatment of acute venous thromboembolism. A randomized, double-blind, controlled trial, Ann Intern Med 138:714, 2003. Kraaijenhagen RA et al: Simplification of the diagnostic management of suspected deep vein thrombosis, Arch Intern Med 162:907, 2002. Meyer G et al: Comparison of low-molecularweight heparin and warfarin for the secondary prevention of venous thromboembolism in patients with cancer, Arch Intern Med 162:1729, 2002. Schulman S et al: Secondary prevention of venous thromboembolism with the oral direct thrombin inhibitor ximelagran, N Engl J Med 349:1713, 2003. Stevens SM et al: Withholding anticoagulation after a negative result on duplex ultrasonography for suspected symptomatic deep venous thrombosis, Ann Intern Med 140:985, 2004. Wells PS et al: Evaluation of d-dimer in the diagnosis of suspected deep vein thrombosis, N Engl J Med 349:1227, 2003. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

•

• Measurement of D-dimer after withdrawal of oral anticoagulation may be useful to estimate the risk of recurrence. Patients with a first spontaneous DVT and a D-dimer level 250 g/ml after withdrawal of oral anticoagulation have a low risk of DVT recurrence.

Section I

•

DVT. Warfarin therapy should be initiated when appropriate (immediately or within 72 hr of initiation of heparin). A 5 mg loading dose of warfarin is recommended in inpatients because it produces less excess anticoagulation than does a 10 mg dose; the smaller dose also avoids the development of a potential hypercoagulable state caused by precipitous decreases in levels of protein C during the first 36 hr of warfarin therapy. In the outpatient setting, a warfarin nomogram using 10 mg loading doses may be more effective in reaching a therapeutic INR. Low–molecular-weight heparin, when used, should be overlapped with warfarin for at least 5 days and until the INR has exceeded 2 for 2 consecutive days. Exclusions from outpatient treatment of DVT include patients with potential high complication risk (e.g., hemoglobin 7, platelet count 75,000, guaiac-positive stool, recent CVA or noncutaneous surgery, noncompliance). Insertion of an inferior vena cava filter to prevent pulmonary embolism is recommended in patients with contraindications to anticoagulation. Thrombolytic therapy (streptokinase) can be used in rare cases (unless contraindicated) in patients with extensive iliofemoral venous thrombosis and a low risk of bleeding.

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BASIC INFORMATION DEFINITION A thyroid nodule is an abnormality found on physical examination of the thyroid gland; nodules can be benign (70%) or malignant. ICD-9CM CODES 241.0 Nodule, thyroid

EPIDEMIOLOGY & DEMOGRAPHICS • Palpable thyroid nodules occur in 4%7% of the population. • Thyroid nodules can be found in 50% of autopsies; however, only 1 in 10 is palpable. • Malignancy is present in 5%-30% of palpable nodules. • Incidence of thyroid nodules increases after 45 yr of age. They are found more frequently in women. • History of prior head and neck irradiation increases the risk of thyroid cancer. • Increased likelihood that nodule is malignant: nodule increasing in size or 2 cm, regional lymphadenopathy, fixation to adjacent tissues, symptoms of local invasion (dysphagia, hoarseness, neck pain, male sex, family history of thyroid cancer or polyposis [Gardner syndrome]).

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Palpable, firm, and nontender nodule in the thyroid area should prompt suspicion of carcinoma. Signs of metastasis are regional lymphadenopathy, inspiratory stridor. • Signs and symptoms of thyrotoxicosis can be found in functioning nodules.

ETIOLOGY • History of prior head and neck irradiation • Family history of pheochromocytoma, carcinoma of the thyroid, and hyperparathyroidism (medullary carcinoma of the thyroid is a component of MENII)

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

Thyroid carcinoma Multinodular goiter Thyroglossal duct cyst Epidermoid cyst Laryngocele Nonthyroid neck neoplasm Branchial cleft cyst

WORKUP • Fine-needle aspiration (FNA) biopsy is the best diagnostic study; the accuracy can be 90%, but it is directly related to the level of experience of the physician and the cytopathologist interpreting the aspirate. • FNA biopsy is less reliable with thyroid cystic lesions; surgical excision should be considered for most thyroid cysts not abolished by aspiration. • A diagnostic approach to thyroid nodule is described in Section III.

LABORATORY TESTS • TSH, T4, and serum thyroglobulin levels should be obtained before thyroidectomy in patients with confirmed thyroid carcinoma on FNA biopsy. • Serum calcitonin at random or after pentagastrin stimulation is useful when suspecting medullary carcinoma of the thyroid and in anyone with a family history of medullary thyroid carcinoma.

IMAGING STUDIES • Thyroid ultrasound is done in some patients to evaluate the size of the thyroid and the number, composition (solid vs. cystic), and dimensions of the thyroid nodule; solid thyroid nodules have a higher incidence of malignancy, but cystic nodules can also be malignant. • The introduction of high-resolution ultrasonography has made it possible to detect many nonpalpable nodules (incidentalomas) in the thyroid (found at autopsy in 30%-60% of cadavers). Most of these lesions are benign. For most patients with nonpalpable nodules that are incidentally detected by thyroid imaging, simple follow-up neck palpation is sufficient. • Thyroid scan with technetium-99m pertechnetate: 1. Classifies nodules as hyperfunctioning (hot), normally functioning (warm), or nonfunctioning (cold); cold nodules have a higher incidence of malignancy. 2. Scan has difficulty evaluating nodules near the thyroid isthmus or at the periphery of the gland. 3. Normal tissue over a nonfunctioning nodule might mask the nodule as “warm” or normally functioning. • Both thyroid scan and ultrasound provide information about the risk of malignant neoplasia based on the characteristics of the thyroid nodule, but their value in the initial evaluation of a thyroid nodule is limited because neither provides a definite tissue diagnosis.

TREATMENT GENERAL Rx • Evaluation of results of FNA 1. Normal cells: may repeat biopsy during present evaluation or reevaluate patient after 3-6 mo of suppressive therapy (l-thyroxine, prescribed in doses to suppress the TSH level to 0.1-0.5) a. Failure to regress indicates increased likelihood of malignancy. b. Reliance on repeat needle biopsy is preferable to routine surgery for nodules not responding to thyroxine. 2. Malignant cells: surgery 3. Hypercellularity: thyroid scan a. Hot nodule: 131I therapy if the patient is hyperthyroid b. Warm or cold nodule: surgery (rule out follicular adenoma vs. carcinoma)

DISPOSITION Variable with results of FNA biopsy.

REFERRAL Surgical referral for FNA biopsy

PEARLS & CONSIDERATIONS COMMENTS • Most solid, benign nodules grow, therefore an increase in nodule volume alone is not a reliable predictor of malignancy. • Surgery is indicated in hard or fixed nodule, presence of dysphagia or hoarseness, and rapidly growing solid masses regardless of “benign” results on FNA. • Suppressive therapy of malignant thyroid nodules postoperatively with thyroxine is indicated. The use of suppressive therapy for benign solitary nodules is controversial.

SUGGESTED READINGS Alexander EK et al: Natural history of benign solid and cystic thyroid nodules, Ann Intern Med 138:315, 2003. Welker MJ, Orlov D: Thyroid nodules, Am Fam Physician 67:559, 2003. AUTHOR: FRED F. FERRI, M.D.

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Tinea Corporis BASIC INFORMATION Tinea corporis is a dermatophyte fungal infection caused by the genera Trichophyton or Microsporum. Ringworm Body ringworm Tinea circinata ICD-9CM CODES 110.5 Tinea corporis

EPIDEMIOLOGY & DEMOGRAPHICS • The disease is more common in warm climates. • There is no predominant age or sex.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Typically appears as single or multiple annular lesions with an advancing scaly border; the margin is slightly raised, reddened, and may be pustular. • The central area becomes hypopigmented and less scaly as the active border progresses outward (Fig. 1-47). • The trunk and legs are primarily involved. • Pruritus is variable.

Trichophyton rubrum is the most common pathogen.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • • • •

Pityriasis rosea Erythema multiforme Psoriasis SLE Syphilis Nummular eczema Eczema Granuloma annulare Lyme disease Tinea versicolor Contact dermatitis

WORKUP Diagnosis is usually made on clinical grounds. It can be confirmed by direct visualization under the microscope of a small fragment of the scale using wet mount preparation and potassium hydroxide solution; dermatophytes appear as translucent branching filaments (hyphae) with lines of separation appearing at irregular intervals.

• Microscopic examination of hyphae • Mycotic culture is usually not necessary • Biopsy is indicated only when the diagnosis is uncertain and the patient has failed to respond to treatment

TREATMENT NONPHARMACOLOGIC THERAPY Affected areas should be kept clean and dry.

ACUTE GENERAL Rx • Various creams are effective; the application area should include normal skin about 2 cm beyond the affected area: 1. Miconazole 2% cream (MonistatDerm) applied bid for 2 wk 2. Clotrimazole 1% cream (Mycelex) applied and gently massaged into the affected areas and surrounding areas bid for up to 4 wk 3. Naftifine 1% cream (Naftin) applied qd 4. Econazole 1% (Spectazole) applied qd • Systemic therapy is reserved for severe cases and is usually given up to 4 wk; commonly used agents: 1. Ketoconazole (Nizoral), 200 mg qd 2. Fluconazole (Diflucan), 200 mg qd 3. Terbinafine (Lamisil), 250 mg qd

DISPOSITION Majority of cases resolve without sequelae within 3-4 wk of therapy.

REFERRAL Dermatology referral in patients with persistent or recurrent infections

SUGGESTED READINGS Friedlander SF et al: Terbinafine in the treatment of trichophytin tinea capitis, Pediatrics 109:602, 2002. Hainer BL: Dermatophyte infections, Am Fam Physician 67:101, 2003. Weinstein A, Berman B: Topical treatment of common superficial tinea infections, Am Fam Physician 65:2095, 2002. AUTHOR: FRED F. FERRI, M.D.

FIGURE 1-47 Annular lesion (tinea corporis). Note raised erythematous scaling border and central clearing. (From Noble J et al: Textbook of primary care medicine, ed 3, St Louis, 2001, Mosby.)

DISEASES AND DISORDERS

SYNONYMS

ETIOLOGY

LABORATORY TESTS Section I

DEFINITION

• It is important to remember that recent topical corticosteroid use can significantly alter the appearance of the lesions.

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Tinea Pedis BASIC INFORMATION DEFINITION Tinea pedis is a dermatophyte infection of the feet.

SYNONYMS Athlete’s foot ICD-CM CODES 110.4 Tinea pedis

• Pruritus is common and is most intense following removal of shoes and socks. • Infection with Tinea rubrum often manifests with a moccasin distribution affecting the soles and lateral feet.

ETIOLOGY Dermatophyte infection caused by T. rubrum, T. mentagrophytes, or less commonly E. floccosum

LABORATORY TESTS • Microscopic examination of a scale or the roof of a blister with 10% KOH under low or medium power will reveal hyphae. • Mycologic culture is rarely indicated in the diagnosis of tinea pedis. • Biopsy is reserved for when the diagnosis remains in question after testing or failure to respond to treatment.

IMAGING STUDIES

EPIDEMIOLOGY & DEMOGRAPHICS

DIAGNOSIS

None

• Most common dermatophyte infection • Increased incidence in hot humid weather. Occlusive footwear is a contributing factor • More common in adult males

DIFFERENTIAL DIAGNOSIS

TREATMENT

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Typical presentation is variable and ranges from erythematous scaling plaques (see Fig. 1-48) and isolated blisters to interdigital maceration. • The infection usually starts in the interdigital spaces of the foot. Most infections are found in the toe webs or in the soles. • Fourth or fifth toes are most commonly involved.

• • • • •

Contact dermatitis Toe web infection Eczema Psoriasis Keratolysis exfoliativa

WORKUP • Diagnosis is usually made by clinical observation. • Laboratory testing, when performed, generally consists of a simple potassium hydroxide (KOH) preparation with mycologic examination under a light microscope to confirm the presence of dermatophytes.

NONPHARMACOLOGIC THERAPY • Keep infected area clean and dry. Aerate feet by using sandals when possible. • Use 100% cotton socks rather than nylon socks to reduce moisture. • Areas likely to become infected should be dried completely before being covered with clothes.

ACUTE GENERAL THERAPY • Butenafine Hcl 1% (Mentax) cream applied bid for 1 wk or qd for 4 wk is effective in interdigital tinea pedis. • Ciclopirox 0.77% (Loprox) cream applied bid for 4 wk is also effective. • Clotrimazole 1% (Lotrimin AF) cream is an OTC treatment. It should be applied to affected and surrounding area bid for up to 4 wk. • Naftifine (Naftin) 1% cream applied qd or gel applied bid for 4 wk also produces a significantly high cure rate. • When using topical preparations, the application area should include normal skin about 2 cm beyond the affected area. • Areas of maceration can be treated with Burow’s solution soaks for 10-20 min bid followed by foot elevation. • Oral agents (fluconazole 150 mg once/wk for 4 wk) can be used in combination with topical agents in resistant cases.

PEARLS & CONSIDERATIONS Combination therapy of antifungal and corticosteroid (clotrimazole/betamethasone [Lotrisone]) should only be used when the diagnosis of fungal infection is confirmed and inflammation is a significant issue. FIGURE 1-48 Tinea pedis. (From Goldstein BG, Goldstein AO: Practical dermatology, ed 2, St Louis, 1997, Mosby.)

SUGGESTED READING Weinstein A, Berman B: Topical treatment of common superficial tinea infections, Am Fam Physician 65:2095, 2002. AUTHOR: FRED F. FERRI, M.D.

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Tinnitus BASIC INFORMATION Tinnitus is the false perception of sound in the absence of an acoustic stimulus.

SYNONYM ICD-9CM CODES 388.30 Tinnitus

EPIDEMIOLOGY & DEMOGRAPHICS • Prevalence: 45-65 yr old: 7% in men, 4% in women; above age 65: 10% in men, 5% in women • More common in whites than in blacks • More common in the southern U.S. • Frequent association with hearing loss SYMPTOMS (ALWAYS SUBJECTIVE) • Ringing (35.5%) • Buzzing (11.2%) • Cricket-like (8.5%) • Hissing (7.8%) • Whistling (6.6%) • Humming (5.3%) • The pitch is high in most cases • Tinnitus is reported to be unilateral (34%), bilateral with lateral dominance (44%), or equal in both ears (22%) • Patients typically wait for several years before seeking medical attention • Most patients report that the tinnitus is much louder subjectively than it is when matched with audible sounds

ETIOLOGY OTOLOGIC: • Noise-induced hearing loss • Presbycusis • Otosclerosis • Otitis • Ceruminosis • Meniere’s disease NEUROLOGIC: • Head and neck injury • Multiple sclerosis • Acoustic neuroma • Other brain tumors

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Objective tinnitus: hearing real sounds 1. Pulsatile sounds: carotid stenosis, aortic valve disease, high cardiac output, arteriovenous malformations 2. Muscular sounds: palatal myoclonus, spasm of stapedius or tensor tympani muscle 3. Spontaneous autoacoustic emissions auditory hallucinations

WORKUP • Description of the sound 1. Constant or episodic 2. Unilateral or bilateral 3. Gradual or sudden onset 4. Duration 5. Hearing loss present or not 6. Vertigo present or not 7. Precipitating factors (e.g., background noise, alcohol, stress, sleep) 8. Impact in daily life PHYSICAL EXAMINATION • Focus on head and neck • Vital signs • Signs of associated illnesses LABORATORY • CBC, FBS, creatinine, ALT, Alk Phos, TSH, lipids, ESR, Lyme titer • Comprehensive audiologic evaluation • In selected cases: brain magnetic resonance imaging with contrast

• Prevent (further) hearing loss with appropriate ear protection and avoidance of noise exposure. • Treat any identified etiologic factor and avoid ototoxic drugs • Medications: 1. Antiarrhythmic drugs (lidocaine, tocainide, flecainide) probably ineffective 2. Benzodiazepines may help, but tinnitus recurs upon cessation of therapy 3. Carbamazepine and other anticonvulsants are ineffective 4. Antidepressants may be helpful and are worth a trial (most studies involve tricyclics) 5. Gingko biloba may be helpful • Acupuncture is ineffective • Tinnitus retraining (habituation) may lead to improvement in as many as 75% of patients. Programs include counseling combined with low-level broadband noise exposure and usually take 1.5 years to complete • Masking devices that cover up the unwanted sounds may be helpful in selected patients • Surgical treatment is controversial • Self-help groups (e.g., the American Tinnitus Association) provide useful information and support • Patient education and reassurance

REFERRAL ENT

SUGGESTED READINGS Lockwood AH, Salvi RJ, Burkard RF: Tinnitus, N Engl J Med 347:904, 2002. Noell CA, Meyeroff WL: Tinnitus: diagnosis and treatment of this elusive symptom, Geriatrics 58:28, 2003. AUTHOR: TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

Ringing in the ear(s)

TREATMENT Section I

DEFINITION

INFECTIONS: • Otitis media • Meningitis • Lyme disease • Syphilis TOXIC (DRUGS): • Aspirin • NSAIDs • Aminoglycosides • Loop diuretics • Vincristine OTHER: • Facial and dental disorders

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BASIC INFORMATION DEFINITION Transient ischemic attack (TIA) refers to a transient neurologic dysfunction caused by focal brain or retinal ischemia with symptoms typically lasting less than 60 min but always less than 24 hr and is followed by a full recovery of function. Acute brain ischemia is a medical emergency requiring prompt neurologic evaluation and potential intervention.

WORKUP

ACUTE GENERAL Rx

• Thorough history and physical examination • Ancillary investigations including neuroimaging aimed at identifying the etiology quickly

• Depends on etiology • If the time of the onset of symptoms is clear, and there are significant deficits on neurologic examination, and brain hemorrhage has been ruled out, then the patient may be a candidate for thrombolytic therapy, but this should be discussed with a neurologist or a specialist in cerebrovascular disease. • Acute anticoagulation: no data supporting benefits in the acute setting. Heparin is considered for new-onset atrial fibrillation and atherothrombotic carotid disease causing recurrent transient neurologic symptoms especially in the setting before carotid endarterectomy or carotid stenting. Also considered for basilar artery thrombosis given concern for progression to brainstem stroke with high morbidity and mortality. • Section III, Transient Ischemic Attacks, describes a treatment algorithm.

LABORATORY TESTS • • • • •

SYNONYMS TIA ICD-9CM CODES 435.9 Unspecified transient cerebral ischemia

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 49 cases/100,000 persons/yr PREDOMINANT SEX: Males  females PEAK INCIDENCE: 60 yr

PHYSICAL FINDINGS & CLINICAL PRESENTATION • During an episode, neurologic abnormalities are confined to discrete vascular territory. • Typical carotid territory symptoms are ipsilateral monocular visual disturbance, contralateral homonymous hemianopsia, contralateral hemimotor or sensory dysfunction, and language dysfunction (dominant hemisphere) alone or in combination. • Typical vertebrobasilar territory symptoms are binocular visual disturbance, vertigo, diplopia, dysphagia, dysarthria, and motor or sensory dysfunction involving the ipsilateral face and contralateral body.

ETIOLOGY • • • •

Cardioembolic Large vessel atherothrombotic disease Lacunar disease Hypoperfusion with fixed arterial stenosis • Hypercoagulable states

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • •

Hypoglycemia Seizures Migraine Subdural hemorrhage Mass lesions Vestibular disease Section II describes the differential diagnosis of neurologic deficits, focal and multifocal.

• • • •

CBC with platelets PT (INR) and PTT Glucose Lipid profile ESR (if clinical suspicion for infectious or inflammatory process) Urinalysis Chest x-ray ECG and consider cycling cardiac enzymes Other tests as dictated by suspected etiology

IMAGING STUDIES • Head CT scan to exclude hemorrhage including a subdural hemorrhage • MRI and MRA. (In several studies, MRI with diffusion-weighted imaging has identified early ischemic brain injury in up to 50% of patients with TIA.) MRA of the brain and neck can identify large vessel intracranial and extracranial stenoses, arteriovenous malformations, and aneurysms • Carotid Doppler studies identify carotid stenosis; neck ultrasound can also visualize stenoses of the vertebrobasilar arteries • Echocardiography if cardiac source is suspected • Telemetry for hospitalized patients for at least 24 hr. May consider 24-hr Holter if patient is being discharged • Four-vessel cerebral angiogram if considering carotid endarterectomy or carotid stent

TREATMENT NONPHARMACOLOGIC THERAPY • Carotid endarterectomy for carotid territory TIA associated with an ipsilateral stenosis of 70%-99%: should be done by a surgeon who is experienced with and performs this procedure frequently. Carotid stenting is also being performed in patients who are not surgical candidates. Trials are comparing stenting versus surgery for carotid disease. • Modification of risk factors including smoking cessation.

CHRONIC Rx • No data supporting the use of long-term anticoagulation in the management of TIA, although stroke patients with atrial fibrillation or demonstrated cardiac thrombi have been shown to benefit from long-term warfarin therapy. • First line of treatment has traditionally been aspirin. No significant benefit of high-dose aspirin (up to 1500 mg/day) has been conclusively found over lower doses (75 mg to 325 mg/day). A baby aspirin (81 mg/day) is therefore appropriate. • Also consider aspirin/dipyridamole extended-release capsules (Aggrenox, 1 capsule po bid) or Plavix as a first-line therapy. In a study of patients with TIA or stroke, Aggrenox reduced subsequent cerebrovascular events to a greater extent than either drug alone. Plavix is equally effective to aspirin in secondary prevention but the combination of aspirin and Plavix for patients with TIA or stroke causes more life threatening bleeding than Plavix alone. Recommend Aggrenox or oral anticoagulation for patients who continue to have TIAs while on aspirin (aspirin failures), but there are no data to support this recommendation. • In patients with cerebrovascular disease, HMG-CoA reductase inhibitors (statins) have been shown to provide significant protection against subsequent vascular events such as MI and stroke even for LDLs 100. Consider starting a statin agent unless LDL is 70.

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Transient Ischemic Attack DISPOSITION

Consider referring patients with TIA for an urgent neurologic evaluation and management.

CAVEAT Urgently evaluate all patients who present with symptoms suggestive of acute brain ischemia. Do not wait for symptoms to resolve to distinguish TIA versus stroke.

SUGGESTED READINGS Alamowitch S et al: Risk, causes, and prevention of ischaemic stroke in elderly patients with symptomatic internal-carotid-artery stenosis, Lancet 357:1154, 2001. Albers GW: A review of published TIA treatment recommendations, Neurology 62:S26, 2004. Albers GW et al: Transient ischemic attack— proposal for a new definition, N Engl J Med 347:1713, 2002. Albers GW et al: Antithrombotic and thrombolytic therapy for ischemic stroke, Chest 119:300S, 2001.

Algra A et al: Oral anticoagulants versus antiplatelet therapy for preventing further vascular events after transient ischaemic attack or minor stroke of presumed arterial origin, Stroke 34:234, 2003. Diener HC et al: Aspirin and clopidrogrel compared with clopidogrel alone after recent ischemic stroke or transient ischaemic attack in high risk patients (MATCH): randomized, double-blind, placebo-controlled trial, Lancet 364:331, 2004. Gorelick PB et al: Prevention of a first stroke, JAMA 281:1112, 1999. Heart Protection Study Collaborative Group: MRC/BHF heart protection study of cholesterol lowering with simvastatin in 20,536 high-risk individuals: a randomized placebocontrolled trial, Lancet 360:7, 2002. Johnston SC: Clinical practice. Transient ischemic attack, N Engl J Med 347:1687, 2002. Sarasin FP, Gaspoz JM, Bounameaux H: Costeffectiveness of new antiplatelet regimens used as secondary prevention of stroke or transient ischemic attack, Arch Intern Med 160(18):2773, 2000. AUTHOR: SEAN I. SAVITZ, M.D.

DISEASES AND DISORDERS

REFERRAL

PEARLS & CONSIDERATIONS

Section I

• According to one study, 10% to 20% of patients have a stroke in the next 90 days, and in 50% of these patients, stroke occurs in the first day or two after the TIA. • Another study showed a stroke risk of 4.4% in the first month and 11.6% in the first year. • The annual risk of myocardial infarction is 2.4%. • One-year and 3-yr survival rates are 98% and 94%, respectively.

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Trigeminal Neuralgia BASIC INFORMATION DEFINITION Trigeminal neuralgia is a syndrome characterized by recurrent excruciating paroxysms of lancinating pain in the distribution of one or more divisions of the trigeminal (fifth) nerve.

SYNONYMS Tic douloureux ICD-9CM CODES 350.1 Trigeminal neuralgia

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): 3-5/100,000 PREVALENCE (IN U.S.): 155/1 million persons PREDOMINANT SEX: Slight predominance of females to males PEAK INCIDENCE: Median age 67 years GENETICS: Uncommonly familial and possibly caused by underlying genetic etiology (see below in “Etiology” under “Rare causes”)

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Each attack lasts only seconds but may cluster. • Often, attacks are brought on by mild stimulation of trigger zones, located in the affected division of the fifth nerve. These triggers include light touching, eating, drinking, shaving, and draft of air.

ETIOLOGY • It is thought that 80%-90% of cases are due to compression of the trigeminal nerve root at the cerebellopontine angle by an aberrant loop of artery or vein, and rarely a saccular aneurysm or arteriovenous malformation • Compressive lesions such as schwannomas, epidermoid cysts, and meningiomas, also typically at the cerebellopontine angle

• Bony compression of the fifth nerve (e.g., from an osteoma or deformity resulting from osteogenesis imperfecta) • Primary demyelinating disorders: multiple sclerosis (2%-4% of patients) and rarely Charcot-Marie-Tooth disease. In multiple sclerosis, usually there is a plaque of demyelination at the root entry zone of the fifth nerve in the pons • Rare causes: (a) infiltrative disorders such as carcinomatous or amyloid deposits in the fifth nerve root, nerve proper, or ganglion; (b) familial occurrence has been reported in CharcotMarie-Tooth disease

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • •

Temporomandibular joint disease Rhinosinusitis Dental pathology The differential diagnosis of headache and facial pain is described in Section II.

WORKUP MRI scans (CT scan with thin posterior fossa cuts if MRI not available) for all patients to exclude mass lesions or evidence of central demyelination as in multiple sclerosis

IMAGING STUDIES See “Workup.”

CHRONIC Rx • Carbamazepine provides relief to at least 75% of patients. Begin with 100 mg bid and increase gradually as tolerated using a tid regimen. • Alternatively may use gabapentin, 400 mg PO tid. Doses as high as 3600 mg/day are easily tolerated, but some patients experience sedation. Topiramate (Topamax) 25 mg qhs gradually titrated up to 100 mg bid is also effective.

DISPOSITION Spontaneous remissions months to years.

occur

after

REFERRAL If uncertain about diagnosis or if surgical treatment is necessary

PEARLS & CONSIDERATIONS Even in patients with multiple sclerosis, a vascular compression may be the source of symptoms and thus may benefit from intervention such as surgery.

COMMENTS Because prolonged remission may occur, drug tapering at yearly intervals is recommended.

SUGGESTED READINGS

ACUTE GENERAL Rx

Elias WJ, Burchiel KJ: Trigeminal neuralgia and other neuropathic pain syndromes of the head and face, Curr Pain Headache Rep 6(2):115, 2002. Kitt CA et al. Trigeminal neuralgia: opportunities for research and treatment, Pain 85:3, 2000 Loeser JD: Tic douloureux, Pain Res Manag 6(3):156, 2001. Love S, Coakham HB: Trigeminal neuralgia: pathology and pathogenesis, Brain 124(Pt 12):2347, 2001. Maesawa S et al: Clinical outcomes after stereotactic radiosurgery for idiopathic trigeminal neuralgia, J Neurosurg 94:16, 2001.

None, episodes are too brief

AUTHOR: U. SHIVRAJ SOHUR, M.D., PH.D.

TREATMENT NONPHARMACOLOGIC THERAPY • In refractory cases, surgical options, including percutaneous radiofrequency gangliolysis and microvascular decompression • Gamma-knife radiosurgery is an increasingly popular alternative to conventional surgery for trigeminal neuralgia

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Trigger Finger (Digital Stenosing Tenosynovitis) • Pain with passive stretching

IMAGING STUDIES

DEFINITION

ETIOLOGY

X-ray studies are not very helpful unless a secondary cause has affected other organs (e.g., rheumatoid lung).

Digital stenosing tenosynovitis refers to an inflammatory process of the digital flexor tendon sheath. Trigger finger ICD-9CM CODES 727.03 Trigger finger (acquired)

EPIDEMIOLOGY & DEMOGRAPHICS • Trigger finger can be found in all age groups but is commonly found in patients older than 45 yr • More frequently affects females (4:1) • Occupational risk groups: meat cutters, seamstresses, tailors, and dentists • In adults the middle finger is most often affected (Fig. 1-49)

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Hand pain • Painful triggering or snapping with flexion and extension of the affected digit • Locking or loss of active digital extension is the most common symptom • The digit possibly fixed in flexion (trapped or incarcerated) • Usually affects one digit • If more digits are involved, a systemic cause most likely present (e.g., diabetes, rheumatoid arthritis) • A palpable tender nodule noted at the MCP joint of the affected digit • Pain over the flexor tendon with resisted flexion

DIAGNOSIS The diagnosis of trigger finger is usually made by the clinical historical presentation and by physical examination.

DIFFERENTIAL DIAGNOSIS • • • • • • •

Dupuytren’s contracture De Quervain’s tenosynovitis Acute digital tenosynovitis Proliferative tenosynovitis Carpal tunnel syndrome Flexion tendon rupture Trauma

WORKUP If a secondary cause of trigger finger is suspected, a workup should be pursued.

LABORATORY TESTS • • • • • •

CBC with differential Electrolytes, BUN, and creatinine Blood glucose Thyroid function tests Uric acid Rheumatoid factor

TREATMENT NONPHARMACOLOGIC THERAPY Splinting can be tried early in the course but has been very successful.

ACUTE GENERAL Rx • In primary idiopathic trigger finger steroid injection, 15-20 mg depomethylprednisolone acetate in 1 ml 1% Xylocaine has been used with success. • Triamcinolone 10 mg with 1 ml of 1% Xylocaine is an alternative steroid choice to be used in patients who do not respond to the first injection. • If symptoms do not resolve in 3 wk, a repeat injection can be tried.

CHRONIC Rx • Surgical release is indicated in patients with refractory symptoms (e.g., locked digits) despite nonpharmacologic and acute treatment. • Surgery is also indicated in patients with recurrent symptoms despite steroid injection therapy.

DISPOSITION • Following steroid injection, symptoms usually resolve in 3-5 days, and locking resolves in 60% of the cases in 2-3 wk. • If symptoms recur, a repeat steroid injection improves the symptoms in 80% of patients. • Diabetic patients do not have the same success rate with steroid injections as the primary idiopathic group.

REFERRAL If steroid injection therapy is considered, a rheumatology consult may be requested.

PEARLS & CONSIDERATIONS COMMENTS If more than one digit is involved, a workup for a secondary systemic cause is in order.

SUGGESTED READINGS

FIGURE 1-49 Trigger finger. Frequency of trigger finger according to digit in adults. (From Canoso J: Rheumatology in primary care, Philadelphia, 1997, WB Saunders.)

Canoso JJ: Trigger finger. In Rheumatology in Primary Care, Philadelphia, 1997, Saunders. Chin DH, Jones NF: Repetitive motion hand disorder, J Calif Dent Assoc 30(2):49, 2002. Moore JS: Flexor tendon entrapment of the digits (trigger finger and trigger thumb), J Occup Environ Med 42(5):526, 2000. Saldana MJ: Trigger digits: diagnosis and treatment, J Am Acad Orthop Surg 9(4):246, 2001. AUTHOR: PETER PETROPOULOS, M.D.

DISEASES AND DISORDERS

SYNONYMS

Trigger finger is described as being primary or secondary: • Primary (idiopathic) • Secondary 1. Diabetes 2. Rheumatoid arthritis 3. Hypothyroidism 4. Histiocytosis 5. Amyloidosis 6. Gout

Section I

BASIC INFORMATION

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Trochanteric Bursitis BASIC INFORMATION DEFINITION Trochanteric bursitis is a presumed inflammation or irritation of the gluteus maximus bursa or the bursa separating the greater trochanter from the gluteus medius and gluteus minimus (Fig. 1-50).

SYNONYMS Greater trochanteric pain syndrome ICD-9CM CODES 726.5 Bursitis trochanteric area

• Trochanteric bursitis can occur when other conditions such as osteoarthritis of the knee and hip and bunions of the feet cause changes in the patient’s gait, placing varus stress on the hip joint.

DIAGNOSIS A detailed physical examination and clinical presentation usually make the diagnosis of trochanteric bursitis. Laboratory tests and x-ray images are helpful adjunctive studies used to exclude other conditions either associated with or mimicking trochanteric bursitis.

EPIDEMIOLOGY & DEMOGRAPHICS

DIFFERENTIAL DIAGNOSIS

• Trochanteric bursitis is commonly associated with other conditions: 1. Osteoarthritis of the hip 2. Lumbar spinal degenerative joint disease 3. Rheumatoid arthritis • Incidence peaks between the fourth and sixth decades of life but can occur at any age group • Occurs in females  males (4:1)

• • • • • • • • • • •

PHYSICAL FINDINGS & CLINICAL PRESENTATION

WORKUP

Osteoarthritis of the hip Osteonecrosis of the hip Stress fracture of the hip Osteoarthritis of the lumbar spine Fibromyalgia Iliopsoas bursitis Trochanteric tendonitis Gout Pseudogout Trauma Neuropathy

• Hip pain is the most common complaint. The pain is chronic, intermittent, and located over the lateral thigh. • Numbness can be present. • Pain is precipitated with prolonged lying or standing on the affected side. • Walking, climbing, and running exacerbate the pain. • Point tenderness over the greater trochanter is noted. • Pain is reproduced with resisted hip abduction.

A workup is indicated if suspected associated conditions exist; otherwise treatment can be started on clinical grounds alone.

ETIOLOGY

• Plain x-rays of the hip are not very helpful in diagnosing trochanteric bursitis. Sometimes calcifications may be seen around the greater trochanter. • Bone scan can be done but is usually not necessary. • CT and MRI may show bursitis but are usually not warranted because it will not alter treatment.

• The specific cause of trochanteric bursitis is not known although repetitive high-intensity use of the hip joint, trauma, infection (tuberculosis and bacterial), and crystal deposition can precipitate the disease.

FIGURE 1-50 Typical location of pain in trochanteric bursitis syndrome. This is also a frequent pain radiation site for lumbar spine lesion, various nerve compression syndromes, and hip disease, particularly in osteonecrosis of the femoral head. (From Canoso J: Rheumatology in primary care, Philadelphia, 1997, WB Saunders.)

LABORATORY TESTS CBC with differential may show elevated white count if infection is present. ESR is elevated in an infectious process. Uric acid may be elevated in patients with gout.

IMAGING STUDIES

TREATMENT NONPHARMACOLOGIC THERAPY • Heat 15-20 min four to six times per day • Ultrasound therapy • Rest • Partial weight bearing • Physical therapy to strengthen back, hip, and knee muscles

ACUTE GENERAL Rx • NSAIDs, ibuprofen 800 mg PO tid, or naproxen 500 mg PO bid is used for pain relief. • Acetaminophen 500-mg tablet, 1-2 tablets PO q6h prn can be used with NSAIDs or alternating with NSAIDs. • Corticosteroid injection (30-40 mg depomethylprednisolone acetate mixed with 3 ml 1% Xylocaine)

CHRONIC Rx Although rarely done, surgical removal of the bursa is possible for patients with refractory symptoms or infection.

DISPOSITION • Most patients respond to NSAIDs and/or nonpharmacologic therapy. • If steroid injection is used, approximately 70% of patients respond after the first injection and more than 90% respond to two injections. • 25% of patients receiving steroid injection may develop a relapse.

REFERRAL A rheumatology or orthopedics referral may be made if steroid injection therapy is needed or if the etiology is thought to be infectious.

PEARLS & CONSIDERATIONS COMMENTS • The absence of pain with flexion and extension differentiates trochanteric bursitis from degenerative joint disease of the hip. • Localization of pain over the lateral thigh differentiates trochanteric bursitis from pain caused by meralgia paresthetica located over the anterolateral thigh and pain from osteoarthritis located over the inner thigh groin area.

SUGGESTED READINGS Adkins SB, Figler RA: Hip pain in athletes, Am Fam Physician 61(7):2109, 2000. Canoso JJ: Hip pain. In Canoso JJ, Kersey R (eds): Rheumatology in primary care. Philadelphia, 1997, WB Saunders. AUTHOR: MEL ANDERSON, M.D.

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Tuberculosis, Pulmonary BASIC INFORMATION •

Pulmonary tuberculosis (TB) is an infection of the lung and, occasionally, surrounding structures, caused by the bacterium Mycobacterium tuberculosis.

•

TB ICD-9CM CODES 011.9 Pulmonary tuberculosis

EPIDEMIOLOGY & DEMOGRAPHICS INCIDENCE (IN U.S.): • Approximately 7 cases/100,000 persons—lowest in reported history • 90% of new cases each year from reactivated prior infections • 9% newly infected • Only 10% of patients with PPD conversions (higher [8%/yr] in HIVpositive patients) will develop TB, most within 1-2 yr • Two thirds of all new cases in racial and ethnic minorities • Occurs most frequently in geographic areas and among populations with highest AIDS prevalence 1. Poor, crowded urban communities • Nearly 36% of new cases from new immigrants PREVALENCE (IN U.S.): • Estimated 10 million people infected • Varies widely among population groups PREDOMINANT SEX: • No specific predilection • Male predominance in AIDS, shelters, and prisons reflected in disproportionate male incidence PREDOMINANT AGE: • Nursing home outbreaks among elderly PEAK INCIDENCE: • Elderly • HIV-positive patients, regardless of age, at highest risk GENETICS: • Populations with widespread low native resistance have been intensely infected when initially exposed to TB. • Following elimination of those with least native resistance, incidence and prevalence of TB tend to decline.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • See “Etiology” • Primary pulmonary TB infection generally asymptomatic • Reactivation pulmonary TB 1. Fever 2. Night sweats 3. Cough 4. Hemoptysis

•

•

ETIOLOGY • Mycobacterium tuberculosis (Mtb), a slow-growing, aerobic, non–sporeforming, nonmotile bacillus, with a lipid-rich cell wall 1. Lacks pigment 2. Produces niacin 3. Reduces nitrate 4. Produces heat-labile catalase 5. Mtb staining, acid-fast, and acidalcohol fast by Ziehl-Neelsen method, appearing as red, slightly bent, beaded rods 2-4 microns long (acid-fast bacilli [AFB]), against a blue background 6. Polymerase chain reaction (PCR) to detect 10 organisms/ml in sputum (compared with the requisite 10,000 organisms/ml for AFB smear detection) 7. Culture a. Growth on solid media (Löwenstein-Jensen; Middlebrook 7H11) in 2-6 wk b. Growth in liquid media (BACTEC, using a radioactive carbon source for early growth detection) often in 9-16 days c. Enhanced in a 5%-10% carbon dioxide atmosphere 8. DNA fingerprinting (based on restriction fragment length polymorphism [RFLP]) a. Facilitates immediate identification of Mtb strains in early growing cultures b. False-negatives possible if growth suboptimal 9. Humans are the only reservoir for Mtb 10. Transmission a. Facilitated by close exposure to high-velocity cough (unprotected by proper mask or respirators) from patient with AFBpositive sputum and cavitary lesions, producing aerosolized droplets containing AFB, which are inhaled directly into alveoli

DISEASES AND DISORDERS

SYNONYMS

b. Occurs within prisons, nursing homes, and hospitals • Pathogenesis 1. AFB (Mtb) ingested by macrophages in alveoli, then transported to regional lymph nodes where spread is contained 2. Some AFB may reach bloodstream and disseminate widely 3. Primary TB (asymptomatic, minimal pneumonitis in lower or midlung fields, with hilar lymphadenopathy) essentially an intracellular infection, with multiplication of organisms continuing for 2-12 wk after primary exposure, until cell-mediated hypersensitivity (detected by positive skin test reaction to tuberculin purified protein derivative [PPD]) matures, with subsequent containment of infection 4. Local and disseminated AFB thus contained by T-cell–mediated immune responses a. Recruitment of monocytes b. Transformation of lymphocytes with secretion of lymphokines c. Activation of macrophages and histiocytes d. Organization into granulomas, where organisms may survive within macrophages (Langhans’ giant cells), but within which multiplication essentially ceases (95%) and from which spread is prohibited 5. Progressive primary pulmonary disease a. May immediately follow the asymptomatic phase b. Necrotizing pulmonary infiltrates c. Tuberculous bronchopneumonia d. Endobronchial TB e. Interstitial TB f. Widespread miliary lung lesions 6. Postprimary TB pleurisy with pleural effusion a. Develops after early primary infection, although often before conversion to positive PPD b. Results from pleural seeding from a peripheral lung lesion or rupture of lymph node into pleural space c. May produce a large (sometimes hemorrhagic) exudative effusion (with polymorphonuclear cells early, rapidly replaced by lymphocytes), frequently without pulmonary infiltrates d. Generally resolves without treatment e. Portends a high risk of subsequent clinical disease, and

Section I

DEFINITION

5. Scanty nonpurulent sputum 6. Weight loss Progressive primary pulmonary TB disease: same as reactivation pulmonary TB TB pleurisy 1. Pleuritic chest pain 2. Fever 3. Shortness of breath Rare massive, suffocating, fatal hemoptysis secondary to erosion of pulmonary artery within a cavity (Rasmussen’s aneurysm) Chest examination 1. Not specific 2. Usually underestimates extent of disease 3. Rales accentuated following a cough (posttussive rales)

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Tuberculosis, Pulmonary

7.

8.

9.

10.

11. 12.

therefore must be diagnosed and treated early (pleural biopsy and culture) to prevent future catastrophic TB illness f. May result in disseminated extrapulmonary infection Reactivation pulmonary TB a. Occurs months to years following primary TB b. Preferentially involves the apical posterior segments of the upper lobes and superior segments of the lower lobes c. Associated with necrosis and cavitation of involved lung, hemoptysis, chronic fever, night sweats, weight loss d. Spread within lung occurs via cough and inhalation Reinfection TB a. May mimic reactivation TB b. Ruptured caseous foci and cavities, which may produce endobronchial spread Mtb in both progressive primary and reactivation pulmonary TB a. Intracellular (macrophage) lesions (undergoing slow multiplication) b. Closed caseous lesions (undergoing slow multiplication) c. Extracellular, open cavities (undergoing rapid multiplication) d. INH and rifampin are cidal in all three sites e. PZA especially active within acidic macrophage environment f. Extrapulmonary reactivation disease also possible Most symptoms (fever, weight loss, anorexia) and tissue destruction (caseous necrosis) from cytokines and cell-mediated immune responses Mtb has no important endotoxins or exotoxins Granuloma formation related to tumor necrosis factor (TNF) secreted by activated macrophages

WORKUP • Sputum for AFB stains • Chest x-ray examination • PPD 1. Recent conversion from negative to positive within 3 mo of exposure is highly suggestive of recent infection. 2. Single positive PPD is not helpful diagnostically. 3. Negative PPD never rules out acute TB. 4. Be certain that positive PPD does not reflect “booster phenomenon” (prior positive PPD may become negative after several years and return to positive only after second repeated PPD; repeat second PPD within 1 wk), which thus may mimic skin test conversion. 5. Positive PPD reaction is determined as follows: a. Induration after 72 hr of intradermal injection of 0.1 ml of 5 TU-PPD b. 5-mm induration if HIV-positive, close contact of active TB, fibrotic chest lesions c. 10-mm induration if in high– medical risk groups (immunosuppressive disease or therapy, renal failure, gastrectomy, silicosis, diabetes), foreign-born highrisk group (Southeast Asia, Latin America, Africa, India), low socioeconomic groups, IV drug addict, prisoner, health care worker d. 15-mm induration if low risk 6. Anergy antigen testing (using mumps, Candida, tetanus toxoid) may identify patients who are truly anergic to PPD and these antigens, but results are often confusing. Not recommended. 7. Patients with TB may be selectively anergic only to PPD. 8. Positive PPD indicates prior infection but does not itself confirm active disease.

LABORATORY TESTS

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • Necrotizing pneumonia (anaerobic, gram-negative) • Histoplasmosis • Coccidioidomycosis • Melioidosis • Interstitial lung diseases (rarely) • Cancer • Sarcoidosis • Silicosis • Paragonimiasis • Rare pneumonias 1. Rhodococcus equi (cavitation) 2. Bacillus cereus (50% hemoptysis) 3. Eikenella corrodens (cavitation)

• Sputum for AFB stains and culture 1. Induced sputum if patient not coughing productively • Sputum from bronchoscopy if high suspicion of TB with negative expectorated induced sputum for AFB 1. Positive AFB smear is essential before or shortly after treatment to ensure subsequent growth for definitive diagnosis and sensitivity testing 2. Consider lung biopsy if sputum negative, especially if infiltrates are predominantly interstitial • AFB stain-negative sputum may grow Mtb subsequently • Gastric aspirates reliable, especially in HIV-negative patients

• CBC 1. Variable values a. WBCs: low, normal, or elevated (including leukemoid reaction: 50,000) b. Normocytic, normochromic anemia often 2. Rarely helpful diagnostically • ESR usually elevated • Thoracentesis 1. Exudative effusion a. Elevated protein b. Decreased glucose c. Elevated WBCs (polymorphonuclear leukocytes early, replaced later by lymphocytes) d. May be hemorrhagic 2. Pleural fluid usually AFB-negative 3. Pleural biopsy often diagnostic— may need to be repeated for diagnosis 4. Culture pleural biopsy tissue for AFB • Bone marrow biopsy is often diagnostic in difficult-to-diagnose cases, especially miliary tuberculosis

IMAGING STUDIES • Chest x-ray examination 1. Primary infection reflected by calcified peripheral lung nodule with calcified hilar lymph node 2. Reactivation pulmonary TB a. Necrosis b. Cavitation (especially on apical lordotic views) c. Fibrosis and hilar retraction d. Bronchopneumonia e. Interstitial infiltrates f. Miliary pattern g. Many of previous may also accompany progressive primary TB 3. TB pleurisy a. Pleural effusion, often rapidly accumulating and massive 4. TB activity not established by single chest x-ray examination 5. Serial chest x-ray examinations are excellent indicators of progression or regression

TREATMENT NONPHARMACOLOGIC THERAPY • Bed rest during acute phase of treatment • High-calorie, high-protein diet to reverse malnutrition and enhance immune response to TB • Isolation in negative-pressure rooms with high-volume air replacement and circulation, with health care provider wearing proper protective 0.5- to 1-micron filter respirators, until three consecutive sputum AFB smears are negative

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Tuberculosis, Pulmonary ACUTE GENERAL Rx

5. Patients with overwhelming pulmonary or extrapulmonary tuberculosis • To pulmonologist for bronchoscopy or pleural biopsy

PEARLS & CONSIDERATIONS COMMENTS • All contacts (especially close household contacts and infants) should be properly tested for PPD conversions during 3 mo following exposure. • Those with positive PPD should be evaluated for active TB and properly treated or given prophylaxis.

SUGGESTED READINGS

• Monthly follow-up by physician experienced in TB treatment • Confirm sensitivity testing and alter treatment appropriately • Frequent sputum samples until culture is negative • Confirm chest x-ray regression at 2 to 3 mo

Benator D et al: Rifapentine and isoniazid once a week versus rifampicin and isoniazid twice a week for treatment of drug susceptible pulmonary tuberculosis in HIVnegative patients: a randomized clinical trial, Lancet 360(9332):528, 2002. Espinal MA et al: Infectiousness of Mycobacterium tuberculosis in HIV-1-infected patients with tuberculosis: a prospective study, Lancet 355(9200):275, 2000. Kanaya AM, Glidden DV, Chambers HF: Identifying pulmonary tuberculosis in patients with negative sputum smear results, Chest 120(2):349, 2001. Karcic AA et al: An elderly woman with chronic knee pain and abnormal chest radiography, Postgrad Med 77(911):600, 2001. Mulder K: Tuberculosis: a case history, Lancet 358(9283):776, 2001. Salazar GE et al: Pulmonary tuberculosis in children in a developing country, Pediatrics 108(2):448, 2001. Small P, Fujiwara P: Management of tuberculosis in the United States, N Engl J Med 345:189, 2001. Tudo G et al: Detection of unsuspected cases of nosocomial transmission of tuberculosis by use of a molecular typing method, Clin Infect Dis 33(4):453, 2001.

REFERRAL

AUTHOR: GEORGE O. ALONSO, M.D.

CHRONIC Rx • Generally not indicated beyond treatment described previously • Prolonged treatment, supervised by infectious disease expert, in a few very complicated infections caused by resistant organisms

DISPOSITION

• To infectious disease expert for: 1. HIV-positive patient 2. Patient with suspected drugresistant TB 3. Patients previously treated for TB 4. Patients whose fever has not decreased and sputum has not converted to negative in 2-4 wk

DISEASES AND DISORDERS

• Monitor for clinical toxicity (especially hepatitis) 1. Patient and physician awareness that anorexia, nausea, RUQ pain, and unexplained malaise require immediate cessation of treatment 2. Evaluation of LFTs a. Minimal SGOT/SGPT elevations without symptoms generally transient and not clinically significant • Preventive treatment for PPD conversion only (infection without disease) 1. Must be certain that chest x-ray examination is negative and patient has no symptoms of TB 2. INH 300 mg daily for 6-12 mo; at least 12 mo if HIV-positive 3. Most important groups: a. HIV-positive b. Close contact of active TB c. Recent converter d. Old TB on chest x-ray examination e. IV drug addict f. Medical risk factor g. High-risk foreign country h. Homeless

Section I

• Compliance (rigid adherence to treatment regimen) chief determinant of success 1. Supervised directly observed therapy (DOT) recommended for all patients and mandatory for unreliable patients • Preferred adult regimen: DOT 1. Isoniazid (INH) 15 mg/kg (max 900 mg)  rifampin 600 mg  ethambutol (EMB) 30 mg/kg (max 2500 mg)  pyrazinamide (PZA) (2 g [50 kg]; 2.5 g [51 to 74 kg]; 3 g [75 kg]) thrice weekly for 6 mo 2. Alternative, more complicated DOT regimens • Rifapentine, a rifampin derivative with a much longer serum half-life, was shown to be as effective when administered weekly (with weekly isoniazid) as conventional regimens for drugsensitive pulmonary tuberculosis in non-HIV-infected patients. • Short-course daily therapy: adult 1. HIV-negative patient: 6 mo total therapy (2 mo INH 300 mg  rifampin 600 mg  EMB 15 mg/kg [max 2500 mg])  PZA (1.5 g [50 kg]; 2 g [51 to 74 kg]; 2.5 g [75 kg]) daily and until smear negative and sensitivity confirmed; then INH  rifampin daily  4 mo 2. HIV-positive patient: 9 mo total therapy (2 mo INH  rifampin  EMB  PZA daily until smear negative and sensitivity confirmed; then INH  rifampin qd  7 mo) 3. Continue treatment at least 3 mo following conversion to negative cultures • Drug resistance (often multiple drug resistance [MDRTB]) increased by: 1. Prior treatment 2. Acquisition of TB in developing countries 3. Homelessness 4. AIDS 5. Prisoners 6. IV drug addicts 7. Known contact with MDRTB • Never add single drug to failing regimen • Never treat TB with fewer than two to three drugs or two to three new additional drugs

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Urinary Tract Infection BASIC INFORMATION

EPIDEMIOLOGY & DEMOGRAPHICS

DEFINITION

INCIDENCE: In adults, 65 yr and older, at least 10% of men and 20% of women have bacteriuria. PATHOGENESIS: • Four major pathways: 1. Ascending from the urethra 2. Lymphatic 3. Hematogenous 4. Direct extension from another organ system • Other risk factors: neurologic diseases, renal failure, diabetes; anatomic abnormalities: bladder outlet obstruction, urethral stricture, vesicoureteral reflux, fistula, urinary diversion, megacystis, and infected stones; age; instrumentation, poor patient compliance, poor hygiene, infrequent voider, douches, and catheters Catheters: All patients who require a long-term Foley catheter eventually develop significant levels of bacteriuria. Treatment is reserved for those individuals who become symptomatic (i.e., leukocytosis, fever, chills, malaise, loss of appetite, etc.). Using prophylactic antibiotics to treat patients who have chronic catheters is to be discouraged because of the risk of acquiring bacteria that are resistant to antibiotic therapy. • Once bacteria reach the urinary tract, three factors determine whether the infection occurs (Box 1-7): 1. Virulence of the microorganism 2. Inoculum size 3. Adequacy of the host defense mechanisms • These factors also determine the anatomic level of the UTI. Urinary Pathogens: In 95% of UTIs the infecting organism is a member of the Enterobacteriaceae, Pseudomonas aeruginosa, or enterococci. In contrast, the or-

Urinary tract infection (UTI) is a term that encompasses a broad range of clinical entities that have in common a positive urine culture. A conventional threshold is growth of 100,000 colony-forming units per ml from a midstream-catch urine sample. In symptomatic patients, a smaller number of bacteria (between 100 and 10,000 colony-forming units per ml of midstream urine) is recognized as an infection.

SYNONYMS UTI ICD-9CM CODES 595.0 Acute cystitis 595.3 Trigonitis 595.2 Chronic cystitis 590.1 Acute pyelonephritis 590.0 Chronic pyelonephritis 590.8 Nonspecific pyelonephritis

CLASSIFICATION FIRST INFECTION: The first documented UTI tends to be uncomplicated and is easily treated. UNRESOLVED BACTERIURIA: UTI in which the urinary tract is not sterilized during therapy. Main causes are bacterial resistance, patient noncompliance with medication, resistance, mixed bacterial infection, rapid reinfection, azotemia, infected stones, Munchausen’s, and papillary necrosis. BACTERIAL PERSISTENCE: UTI in which the urine cultures become sterile during therapy, but a persistent source of infection from a site within the urinary tract that was excluded from the high urinary concentrations gives rise to reinfection by the same organism. Causes: infected stone, chronic bacterial prostatitis, atrophic infected kidney, vesicovaginal or enterovesical fistulas, obstructive uropathy, infected pyelocalyceal diverticula, infected ureteral stump following nephrectomy, infected necrotic papillae from papillary necrosis, infected urachal cysts, infected medullary sponge kidney, urethral diverticula, and foreign bodies. REINFECTION: UTI in which a new infection occurs with new pathogens at variable intervals after a previous infection has been eradicated. Relapse: The less common form of recurrent infection; occurs within 2 wk of treatment when the same organism reappears in the same site as the previous infection. Relapsing infections of the urinary tract most commonly occur in pyelonephritis, kidney obstruction from a stone, and prostatitis.

BOX 1-7

ganisms that commonly colonize the distal urethra and skin of both men and women and the vagina of women are Staphylococcus epidermidis, diphtheroids, lactobacilli, Gardnerella vaginalis, and a variety of anaerobes that rarely cause UTI. Generally, the isolation of two or more bacterial species from a urine culture signifies a contaminated specimen, unless the patient is being managed with an indwelling catheter or urinary diversion or has a chronic complicated infection. Defense Mechanisms Against Cystitis: Low pH and high osmolarity, mucopolysaccharide glycosaminoglycan protective layer, normal bladder that empties completely and has no incontinence, and the presence of estrogen

PHYSICAL FINDINGS • UTI presentation is inconsistent and cannot be relied upon to diagnose UTI accurately or to localize the site of infection. Patients complain of: 1. Urinary frequency, urgency 2. Dysuria 3. Urge incontinence 4. Suprapubic pain 5. Gross or microscopic hematuria • When negative cultures are associated with significant pyuria, vaginal discharge, or hematuria, infections with Chlamydia trachomatis, Neisseria gonorrhoeae, and Trichomonas vaginalis should be considered. • Acute pyelonephritis (PN) presents with fever, flank or abdominal pain, chills, malaise, vomiting, and diarrhea. It is these systemic symptoms that distinguish pyelonephritis from cystitis. Complications of acute pyelonephritis are renal abscess, perinephric abscess, emphysematous pyelonephritis, and pyonephrosis.

Bacterial Factors

1. The size of the inoculum 2. The virulence of the infecting organism: a. Virulence factors: i. P-fimbriae facilitate the adherence of bacteria to biologic surfaces. ii. K-antigens facilitate adherence and protect the organisms from the hostimmune response. iii. O-antigens are an important source of the systemic reactions, such as fever and shock, that occur with bacterial infections. iv. H antigens are associated with flagella and are related to bacterial locomotion. v. Hemolysin may potentiate tissue damage and facilitate local bacterial growth. vi. Urease alkalinizes the urine and facilitates stone formation, thus potentiating infection. b. Biofilms harbor bacteria on prosthetic devices and may be a source of recurrent infections. c. The presence of sialosyl galactosyl globoside (SGG) on the surface of kidney cells. This compound is a highly powerful receptor for E. coli bacteria. d. Women with a deficiency in human beta-defensin-1 (HBD-1) are at greater risk for urinary tract infection. 3. Adequacy of host defense mechanisms

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Urinary Tract Infection DIAGNOSIS

LABORATORY TESTS • Urinalysis with microscopic evaluation of clean-catch urine for bacteria and pyuria • Urine C&S • CBC with differential (shows leukocytosis) • Antibody-coated bacteria are seen with pyelonephritis

IMAGING STUDIES • Warranted only if renal infection or genitourinary abnormality is suspected • KUB, VCUG, renal sonogram, IVP, CT scan, and nuclear scan • Specialty examination: cystoscopy with occasional retrograde pyelography to rule out obstructive uropathy; stenting the obstruction possibly required

TREATMENT NONPHARMACOLOGIC THERAPY • Hot sitz baths, anticholinergics, urinary analgesics • For pyelonephritis: bed rest, analgesics, antipyretics, and IV hydration

ACUTE GENERAL Rx • Conventional therapy of 7 days; shortterm therapy of 1, 3, or 5 days is not recommended for geriatric patients. • Agents of choice: amoxicillin/clavulanate, cephalosporins, fluoroquinolones, nitrofurantoin, and trimethoprim with sulfonamide. • For pyelonephritis: hospitalization until afebrile and stable, then at home via home care agency with IV antibiotic composed of aminoglycoside plus cephalosporin  1 wk followed by oral agents (based on sensitivity) for 2 wk. Moderate forms of pyelonephritis have been successfully treated with fluoroquinolone therapy for 21 days, without requiring hospitalization. Most important, complicating factors such

PEARLS & CONSIDERATIONS COMMENTS • Asymptomatic bacteriuria: occurs in both anatomically normal and abnormal urinary tracts. Common among nursing home residents. This can clear spontaneously, persist, or lead to symptomatic kidney infection. Treatment is recommended in patients with vesicoureteral reflux, stones, obstructive uropathy, parenchymal renal disease, diabetes mellitus, and immunocompromised patients. • Urinary incontinence and “asymptomatic” bacteriuria often coexist among nursing home patients, and antibiotics rarely correct the incontinence, especially in the absence of pyuria. • UTI can present as delirium, particularly in patients with baseline cognitive impairments. • Recurrent UTI: caused by an unresolved infection, vaginal colonization of the originally infecting organism, or reinfection with a new strain. Management of recurrent UTI includes continuous antibiotic prophylaxis, intermittent self-treatment, and postcoital prophylaxis. Prophylaxis is considered for women who experience two or more symptomatic UTIs over a 6-mo period or three or more episodes over a 12-mo period. 1. Changes after menopause: lower levels of lactobacilli, decreased estrogen, senile atrophy of the genitalia, and loss of bladder elasticity (compliance). 2. Biologic factors altering defense systems: the presence of sialosyl galactosyl globoside (SGG) on the surface of the kidney acts as a powerful receptor for E. coli and increases the risk for UTI; the presence of the blood group P1 causes increased binding of E. coli that is resistant to normal infection-fighting mechanisms in the body and it is believed that some individuals are deficient in a compound called human beta-defensin-1 (HBD-1), a naturally occurring antibiotic that fights E. coli within the urinary tract. 3. BPH and chronic prostatitis in men

SUGGESTED READINGS Bent S et al: Does this woman have an acute uncomplicated urinary tract infection? JAMA 287:2701, 2002. Gomolin IH et al: Efficacy and safety of ciprofloxacin oral suspension versus trimethoprim-sulfamethoxazole oral suspension for treatment of older women with acute urinary tract infection, J Am Geriatr Soc 49:1606, 2001. Gupta K et al: Increasing antimicrobial resistance and the management of uncomplicated community-acquired urinary tract infections, Ann Int Med 135:41, 2001. Levy SB: Multidrug resistance—a sign of the times, N Engl J Med 338:1376, 1998 [editorial]. McIsaac WJ et al: The impact of empirical management of acute cystitis on unnecessary antibiotic use, Arch Int Med 161:600, 2002. Saint S et al: The effectiveness of a clinical practical guideline for the management of presumed uncomplicated UTI in women, Am J Med 106:636, 1999. AUTHORS: PHILIP J. ALIOTTA, M.D., M.S.H.A., with revisions by TOM J. WACHTEL, M.D.

DISEASES AND DISORDERS

• Interstitial cystitis • Vaginitis • Urethritis (gonococcal, nongonococcal, Trichomonas) • Frequency-urgency syndrome, prostatitis (acute and chronic) • Obstructive uropathy • Infected stones • Fistulas • Papillary necrosis • Vesicoureteral reflux

Resistance: • Because of the overuse of antibiotics, organisms once sensitive to a number of agents are now increasingly more resistant, making effective management of UTI and pyelonephritis more difficult and potentially more dangerous. Most important has been the increasing resistance to TMP-SMX, the current primary care provider drug of choice for acute uncomplicated UTI in women. • Facts about bacterial resistance: 1. Given enough antibiotic and time, resistance will develop 2. Organisms that are resistant to one antibiotic will likely become resistant to others 3. Resistance is progressive, moving from low to intermediate to high levels 4. Once selected, drug resistance will not disappear 5. When antibiotics are used by any patient, this use affects other people by changing the immediate and extended environment 6. No counterselective steps against resistant bacteria now exist • When choosing a treatment regimen physicians should consider such factors as: 1. In-vitro susceptibility 2. Adverse effects 3. Cost effectiveness 4. Resistance rates in the respective communities

Section I

DIFFERENTIAL DIAGNOSIS

as obstructive uropathy or infected stones must be identified and treated. • Section III, “Urinary Tract Infection,” describes an approach to the management of UTI.

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Urolithiasis BASIC INFORMATION DEFINITION Urolithiasis is the presence of calculi within the urinary tract. The five major types of urinary stones are calcium oxalate (50%), calcium phosphate (10% to 20%), uric acid (8%), struvite (15%), and cystine (3%).

SYNONYMS Nephrolithiasis Renal colic ICD-9CM CODES 592.9 Urinary calculus

EPIDEMIOLOGY & DEMOGRAPHICS • Urinary stone disease afflicts 250,000 to 750,000 Americans/yr. • Male:female ratio is 4:1. After the sixth decade, the ratio is 1.5:1. • Incidence of symptomatic nephrolithiasis is greatest during the summer (resulting from increased humidity and temperatures with increased risk of dehydration and concentrated urine). • Calcium oxalate or mixed calcium oxalate/calcium phosphate stones account for 70% of urolithiasis.

PHYSICAL FINDINGS & CLINICAL PRESENTATION Stones may be asymptomatic or may cause the following signs and symptoms from obstruction: • Sudden onset of flank tenderness • Nausea and vomiting • Patient in constant movement, attempting to lessen the pain (patients with an acute abdomen are usually still because movement exacerbates the pain) • Pain may be referred to the testes or labium (progression of stone down the urinary ureter)

BOX 1-8

• Fever and chills accompanying the acute colic if there is superimposed infection • Pain may radiate anteriorly over to the abdomen and result in intestinal ileus

ETIOLOGY • Increased absorption of calcium in the small bowel: type I absorptive hypercalciuria (independent of calcium intake) • Idiopathic hypercalciuria nephrolithiasis is the most common diagnosis for patients with calcium stones; the diagnosis is made only if there is no hypercalcemia and no known cause for hypercalciuria • Increased vitamin D synthesis (e.g., secondary to renal phosphate loss: type III absorptive hypercalciuria) • Renal tubular malfunction with inadequate reabsorption of calcium and resulting hypercalciuria • Heterozygous mutations in the NPT2a gene result in hypophosphatemia and urinary phosphate loss • Hyperparathyroidism with resulting hypercalcemia • Elevated uric acid level (metabolic defects, dietary excess) • Chronic diarrhea (e.g., inflammatory bowel disease) with increased oxalate absorption • Type I (distal tubule) renal tubular acidosis (1% of calcium stones) • Chronic hydrochlorothiazide treatment • Chronic infections with ureaseproducing organisms (e.g., Proteus, Providencia, Pseudomonas, Klebsiella). Struvite, or magnesium ammonium phosphate crystals, are produced when the urinary tract is colonized by bacteria, producing elevated concentrations of ammonia • Abnormal excretion of cystine • Chemotherapy for malignancies

DIAGNOSIS DIFFERENTIAL DIAGNOSIS • • • • • • • • •

Urinary tract infection Pyelonephritis Diverticulitis PID Ovarian pathology Factitious (drug addicts) Appendicitis Small bowel obstruction The differential diagnosis of obstructive uropathy is described in Section II

WORKUP • Laboratory and imaging studies. Stone analysis should be performed on recovered stones. • A clinical algorithm for evaluation of nephrolithiasis is described in Section III. • Box 1-8 describes past medical history significant for urolithiasis.

LABORATORY TESTS • Urinalysis: hematuria may be present; however, its absence does not exclude urinary stones. Evaluation of urinary pH is of value in identification of type of stone (pH 7.5 is associated with struvite stones, whereas pH 5 generally is seen with uric acid or with cystine stones). • Urine C&S should be obtained for all patients. • Serum chemistries should include calcium, electrolytes, phosphate, and uric acid. • Additional tests: 24-hr urine collection for calcium, uric acid, phosphate, oxalate, and citrate excretion is generally reserved for patients with recurrent stones.

Past Medical History Significant for Urolithiasis

Diseases associated with disturbances of calcium metabolism: primary hyperparathyroidism, Wilson’s disease, medullary sponge kidney, osteoporosis, immobilization, sarcoidosis, osteolytic metastases, plasmacytoma, neuroendocrine tumors, Paget’s disease Dietary history: purine gluttony, calcium excess, milk alkali, oxalate excess, sodium excess, low citrus fruit intake Medications: uricosurics, diuretics, analgesics, vitamins C and D, antacids (especially phosphorus-binding agents), acetazolamide, calcium channel blockers, triamterene, theophylline, protease inhibitors (indinavir), sulfonamides Diseases associated with disturbances of oxalate metabolism: primary hyperoxaluria types I and II, Crohn’s disease, ulcerative colitis, intestinal bypass surgery (especially jejunoileal bypass), ileal resection Diseases associated with disturbances of purine metabolism Intrinsic metabolic disorders—anemia, neoplastic disorders (especially leukemias), intoxication, myocardial infarction, irradiation, cytotoxic chemotherapy Enzyme deficiency—primary gout, Lesch-Nyhan syndrome Altered excretion—renal insufficiency, metabolic acidosis Infectious history: organisms (particularly Proteus and Klebsiella), febrile upper tract involvement and dates if hospitalized. From Nseyo UO (ed): Urology for primary care physicians, Philadelphia, 1999, WB Saunders.

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Urolithiasis IMAGING STUDIES

NONPHARMACOLOGIC THERAPY • Increase in water or other fluid intake (doubling of previous fluid intake unless patient has a history of CHF or fluid overload) • Normal dietary calcium intake. If one does not consume enough calcium, less is available to bind to dietary oxalate; as a result, more oxalate reaches the colon, is absorbed into the bloodstream, and is excreted as calcium oxalate, setting the stage for calcium urolithiasis • Sodium restriction (to decrease calcium excretion), decreased protein intake to 1 g/kg/day (to decrease uric acid, calcium, and oxalate excretion) • Increase in bran (may decrease bowel transit time with increased binding of calcium and subsequent decrease in urinary calcium)

ACUTE GENERAL Rx • Pain control (use of narcotics is generally indicated because of the severity of pain) • Specific therapy tailored to the stone type: 1. Uric acid calculi: control of hyperuricosuria with allopurinol 100-300 mg/day; increase urinary pH with potassium citrate, 10-mEq tablets tid 2. Calcium stones: a. HCTZ 25-50 mg qd in patients with type I absorptive hypercalciuria b. Decrease bowel absorption of calcium with cellulose phosphate 10 g/day in patients with type I absorptive hypercalciuria

CHRONIC Rx Maintenance of proper hydration and dietary restrictions (see “Acute General Rx”)

DISPOSITION • 50% of patients will pass the stone within 48 hr. • Stones will recur in approximately 50% of patients within 5 yr if no medical treatment is provided.

REFERRAL Urology referral in complicated or recurrent urolithiasis; most patients with small uncomplicated ureteral or renal calculi can be followed as outpatient, whereas patients with persistent vomiting, suspected UTI, pain unresponsive to oral analgesics, or obstructing calculus associated with solitary kidney should be admitted to a hospital

PEARLS & CONSIDERATIONS COMMENTS • Early identification and aggressive treatment of urinary tract infections is indicated in all patients with struvite stones. • Alkalinization of urine (pH 7.5 with penicillamine) is useful in patients with recurrent cystine stones. • An algorithmic approach to the management of ureteral calculi is described in Section III.

SUGGESTED READINGS Borghi L et al: Comparison of two diets for the prevention of recurrent stones in idiopathic hypercalciuria, N Engl J Med 346:77, 2002. Prie D et al: Nephrolithiasis and osteoporosis associated with hypophosphatemia caused by mutations in the type 2a sodium-phosphate cotransporter, N Engl J Med 347:983, 2002. Worster A et al: The accuracy of noncontrast helical computed tomography versus intravenous pyelography in the diagnosis of suspected acute urolithiasis: a meta-analysis, Ann Intern Med 40:280, 2002. AUTHOR: FRED F. FERRI, M.D.

DISEASES AND DISORDERS

TREATMENT

c. Orthophosphates to inhibit vitamin B synthesis in patients with type III absorptive hypercalciuria d. Potassium citrate supplementation in patients with hypocitraturic calcium nephrolithiasis e. Purine dietary restrictions or allopurinol in patients with hyperuricosuric calcium nephrolithiasis 3. Struvite stones: a. Most of the stones are large and cause obstruction and bleeding. b. ESWL and percutaneous nephrolithotomy are generally necessary. c. Prolonged use of antibiotics directed against the predominant urinary tract organism may be beneficial to prevent recurrence. 4. Cystine stones: Hydration and alkalization of the urine to pH 6.5, penicillamine, and tiopronin can also be used to reduce the formation of cystine; captopril is also beneficial and causes fewer side effects • Surgical treatment in patients with severe pain unresponsive to medication and patients with persistent fever or nausea or significant impediment of urine flow: a. Ureteroscopic stone extraction b. Extracorporeal shock wave lithotripsy (ESWL) for most renal stones • In 1997 the American Urological Association issued the following guidelines for the treatment of ureteral stones: 1. Proximal ureteral stones 1 cm in diameter: options are ESWL, percutaneous nephroureterolithotomy, and ureteroscopy 2. Proximal ureteral stones 1 cm in diameter: options are ESWL, percutaneous nephroureterolithotomy, and ureteroscopy. Placement of a ureteral stent should be considered if the stone is causing high-grade obstruction 3. Distal ureteral stones 1 cm in diameter: most of these pass spontaneously. ESWL and ureteroscopy are two accepted modes of therapy 4. Distal ureteral stones 1 cm in diameter: watchful waiting, ESWL, ureteroscopy (following stone fragmentation) • Section III describes an approach to the management of ureteral calculi.

Section I

• Plain films of the abdomen can identify radioopaque stones (calcium, uric acid stones). • Renal sonogram may be helpful. • IVP demonstrates the size and location of the stone, as well as degree of obstruction. • Unenhanced (noncontrast) helical CT scan does not require contrast media and can visualize the calculus (identified by the “rim sign” or “halo” representing the edematous ureteral wall around the stone). It is fast, accurate (sensitivity 15%-100%, specificity 94%96%), and readily identifies all stone types in all locations. This modality is being used increasingly in the initial assessment of renal colic.

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Urticaria BASIC INFORMATION

• Food additives (e.g., salicylates, benzoates, sulfites) • Infections (viral infections, fungal infections, chronic bacterial infections) • Physical stimuli (e.g., pressure urticaria, exercise-induced, solar urticaria, cold urticaria) • Inhalants (e.g., mold spores, animal danders, pollens) • Contact (nonimmunologic) urticaria (e.g., caterpillars, plants) • Other: hereditary angioedema, urticaria pigmentosa, cold urticaria, hair bleaches, chemicals, saliva, cosmetics, perfumes, pemphigoid, emotional stress

ACUTE GENERAL Rx

DIAGNOSIS

• Oral antihistamines: use of nonsedating antihistamines (e.g., loratadine [Claritin] 10 mg qd or cetirizine [Zyrtec] 10 mg qd) is preferred over first-generation antihistamines (e.g., hydroxyzine, diphenhydramine). • Doxepin (a tricyclic antidepressant) that blocks both H1 and H2 receptors 25-75 mg qhs may be effective in patients with chronic urticaria. • H2 receptor antagonists (cimetidine, ranitidine, famotidine) can be added to H1 antagonists in refractory cases. • A short course of rapidly tapering oral corticosteroids may be prescribed (e.g., prednisone starting at 60 mg qd and ending at 10 mg qd over 8 days).

DIFFERENTIAL DIAGNOSIS

CHRONIC Rx

• At least 20% of the population will have one episode of hives during their lifetime. • Incidence is increased in atopic patients. • The etiology of chronic urticaria (hives lasting longer than 6 wk) is determined in only 5%-20% of cases.

• • • • • • •

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• It is useful to determine whether hives are acute or chronic; a medical history focused on various etiologic factors is necessary before embarking on extensive laboratory testing. • A diagnostic approach to chronic urticaria is described in Section III.

• Use of nonsedating antihistamines, doxepin, and/or oral corticosteroids (see “Acute General Rx”) • Oral corticosteroids should be reserved for refractory cases (e.g., prednisone 20 mg qd or 20 mg bid). • Low dose of the immunosuppressant cyclosporine (2.5-3 mg/kg body weight/day) has been shown to be effective and corticosteroid sparing in chronic urticaria • There is insufficient data to support use of leukotriene antagonists (zafirlukast, montelukast) in patients with chronic urticaria

DEFINITION Urticaria is a pruritic rash involving the epidermis and the upper portions of the dermis, resulting from localized capillary vasodilation and followed by transudation of protein-rich fluid in the surrounding tissue and manifesting clinically with the presence of hives.

SYNONYMS Hives Wheals ICD-9CM CODES 708.8 Other unspecified urticaria

EPIDEMIOLOGY & DEMOGRAPHICS

• Presence of elevated, erythematous, or white nonpitting plaques that change in size and shape over time; they generally last a few hours and disappear without a trace. • Annular configuration with central pallor (Fig. 1-51).

ETIOLOGY • Foods (e.g., shellfish, eggs, strawberries, nuts) • Drugs (e.g., penicillin, aspirin, sulfonamides) • Systemic diseases (e.g., SLE, serum sickness, autoimmune thyroid disease, polycythemia vera)

Erythema multiforme Erythema marginatum Erythema infectiosum Urticarial vasculitis Drug eruption Multiple insect bites Bullous pemphigoid

WORKUP

LABORATORY TESTS • CBC with differential • Stool for ova and parasites in patients with suspected parasitic infestations • ANA, ESR, TSH, LFTs, eosinophil count are indicated only in selected patients • Measurement of C4 in patients who present with angioedema alone • Skin biopsy is helpful in patients with fever, arthralgias, and elevated ESR

TREATMENT NONPHARMACOLOGIC THERAPY • Remove suspected etiologic agents (e.g., stop aspirin and all nonessential drugs), restrict diet (e.g., elimination of tomatoes, nuts, eggs, shellfish). • Elimination of yeast should be attempted in patients with chronic urticaria (Candida albicans sensitivity may be a factor in patients with chronic urticaria).

FIGURE 1-51 Wheal (urticaria). Note central cleaning, giving annular configuration. (From Noble J et al: Textbook of primary care medicine, ed 3, St Louis, 2001, Mosby.)

DISPOSITION • Most cases of urticaria resolve within 6 wk. • Only 25% of patients with a history of chronic urticaria are completely cured after 5 yr.

PEARLS & CONSIDERATIONS COMMENTS • Local treatment (e.g., starch baths or Aveeno baths) may be helpful in selected patients; however, local treatment is generally not rewarding. • The management of angioedema (i.e., Dx and Rx) is the same as urticaria.

SUGGESTED READINGS Kaplan AP: Chronic urticaria and angioedema, N Engl J Med 346:157, 2002. Miller BA: Urticaria and angioedema: a practical approach, Am Fam Physician 69:1123, 2004. AUTHORS: FRED F. FERRI, M.D., with minor revisions by TOM J. WACHTEL, M.D.

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Uterine Prolapse (Pelvic Organ Prolapse) BASIC INFORMATION

SYNONYMS Genital prolapse Uterine descensus Pelvic organ prolapse ICD-9CM CODES 618.8 Genital prolapse 618.1 Uterine descensus 618.8 Pelvic organ prolapse

EPIDEMIOLOGY & DEMOGRAPHICS Most prevalent in postmenopausal multiparous women. RISK FACTORS: • Labor • Vaginal childbirth • Obesity • Chronic coughing • Constipation • Pelvic tumors • Ascites • Strenuous physical exertion • Caucasian race GENETICS: Increased incidence in women with spina bifida occulta.

PHYSICAL FINDINGS & CLINICAL PRESENTATION • • • • • • • • • •

Pelvic pressure Bearing-down sensation Bilateral groin pain Sacral backache Coital difficulty Protrusion from vagina Spotting Ulceration Bleeding Examination of patient in lithotomy, sitting, and standing positions and before, during, and after a maximum Valsalva effort • Erosion or ulceration of the cervix possible in the most dependent area of the protrusion

ETIOLOGY • Vaginal childbirth and chronic increases in intraabdominal pressure leading to detachments, lacerations, and denervations of the vaginal support system

DIAGNOSIS

NONPHARMACOLOGIC THERAPY

DIFFERENTIAL DIAGNOSIS • Occasionally, elongated cervix; body of the uterus remains undescended • Diagnosis is based on history and physical examination. Currently there is only one genital tract prolapse classification system that has attained international acceptance & recognition, the Patient pelvic organ prolapse quantification (POPQ). See Boxes 1-9 and 1-10.

WORKUP • If erosion or ulceration of the cervix is present, a Pap smear followed by a cervical biopsy should be performed if indicated. • If urinary symptoms are significant, further urodynamic workup is indicated, looking for concurrent cystourethrocele, cystocele, enterocele, or rectocele.

LABORATORY TESTS Urinalysis and urine culture if urinary symptoms exist

Ultrasound if concurrent fibroids need further evaluation

TREATMENT

• Prophylactic measures 1. Diagnosis and treatment of chronic respiratory and metabolic disorders 2. Correction of constipation 3. Weight control, nutrition, and smoking cessation counseling 4. Teaching of pelvic muscle exercises • Supportive pessary therapy 1. Ring-type pessary useful for first- or second-degree prolapse 2. Gellhorn pessary preferred for more advanced prolapse 3. Use of pessaries in conjunction with continuous hormone replacement therapy, unless contraindicated 4. Perineorrhaphy under local anesthesia possibly needed to support the pessary if the vaginal outlet is very relaxed

ACUTE GENERAL Rx • Patients who are only infrequently symptomatic: insertion of a tampon or diaphragm for temporary relief when prolonged standing is anticipated

Staging of Pelvic Organ Prolapse Based on POP-Q Examination BOX 1-9

Stage Stage Stage Stage

0 I II III

Stage IV

No prolapse. Most distal prolapse more than 1 cm above hymenal ring. Most distal point is 1 cm or less above hymenal ring. Most distal point is more than 1 cm below the hymenal ring but not further than 2 cm less the total vaginal length, i.e.,  1 cm but   (TVL-2) cm. Complete vaginal eversion.

From Pemberton J (ed): The pelvic floor, Philadelphia, 2002, WB Saunders.

BOX 1-10

Points of Reference for POP-Q

Point A Three cm above the hymen on anterior vaginal wall (Aa) or posterior vaginal wall (Ap). Point Aa roughly corresponds with the urethrovesical junction. These points can range from 3 cm (no prolapse) to 3 cm (maximal prolapse.) Point B The lowest extent of the segment of vagina between point A and the apex of the vagina. Unlike point A they are not fixed but will be the same as A if point A is the most protruding point. In maximal prolapse it will be the same as point C. Point C The most distal part of the cervix or vaginal vault. Point D The posterior fornix, which is thus omitted in women with prior hysterectomy. Genital hiatus From midline external urethral meatus to inferior hymenal ring. Perineal body From inferior hymenal ring to middle of anal orifice. Vaginal length This should be measured without undue stretching of the vagina. From Pemberton J (ed): The pelvic floor, Philadelphia, 2002, WB Saunders.

DISEASES AND DISORDERS

Uterine prolapse refers to the protrusion of the uterus into or out of the vaginal canal. In a first-degree uterine prolapse, the cervix is visible when the perineum is depressed. In a second-degree uterine prolapse, the uterine cervix has prolapsed through the vaginal introitus, with the fundus remaining within the pelvis proper. In a third-degree uterine prolapse (i.e., complete uterine prolapse, uterine procidentia), the entire uterus is outside the introitus.

IMAGING STUDIES Section I

DEFINITION

• Further weakening of pelvic support system by hypoestrogenic atrophy • Some cases from congenital or inherited weaknesses within the pelvic support system

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Uterine Prolapse (Pelvic Organ Prolapse) CHRONIC Rx • Hormone replacement therapy at the time of menopause helps preserve tissue strength, maintain elasticity of the vagina, and promote the durability of surgical repairs; however, the routine use of HRT is no longer recommended. • Gold standard for therapy is vaginal hysterectomy. • Vaginal apex should be well suspended, but a prophylactic sacrospinous ligament fixation is not routinely required. • If occult enterocele present, McCall culdoplasty is performed. • If vaginal approach to hysterectomy is contraindicated, abdominal hysterectomy is performed; vaginal apex likewise well supported.

• Colpocleisis is considered for the elderly patient who is sexually inactive and is a high-risk patient from a surgical point of view; can be done rapidly under local anesthesia with mild sedation if necessary. • Other surgical options are sling operations and sacral cervicopexy.

PEARLS & CONSIDERATIONS COMMENTS Surgery contraindicated in mild or asymptomatic uterine prolapse because the patient will seldom benefit from the operation although exposed to its risks.

DISPOSITION If untreated, uterine prolapse progressively worsens.

REFERRAL To a gynecologist/urologist if pessary fitting or surgical intervention is needed

SUGGESTED READINGS Glass RH, Curtis MG, Hopkins MP: Glass’ Office Gynecology, ed 5, Baltimore, 1999, Lippincott Williams & Wilkins. Thaker R: Management of uterine prolapse, BMJ 324:1258, 2002. AUTHOR: ARUNDATHI G. PRASAD, M.D.

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Vision Loss BASIC INFORMATION

SYNONYMS Low vision Blindness ICD-9CM CODES 368.11 Sudden visual loss 368.12 Transient visual loss 369.00 Visual impairment level not further specified 369.3 Unqualified visual loss, both eyes

EPIDEMIOLOGY & DEMOGRAPHICS • 22 million blind people worldwide are over 60 years old. • 17% of Americans 65 to 74 years and 26% of Americans 75 years and older report vision impairment. • 1 in 3 Americans over 75 years has signs of macular degeneration, which is the most common cause of blindness in the U.S. • Glaucoma and cataracts are the most common causes of blindness among African Americans. • 46% of people 75 to 85 years old have cataracts, with a higher prevalence in females. • Patients with visual impairment have an average hospital length of stay 2.4 days longer than those without impairment.

NORMAL & ABNORMAL CHANGES IN VISION WITH AGING Eyelids • Thinning, dehydration, dermatochalasis (redundant skin), ectropion (eversion of the lower lid), entropion (inversion of the lid, causing corneal irritation from the lower lashes) • Site of neoplasms, such basal cell carcinoma and squamous cell carcinoma Tears • Decreased production and thinning of the tear film • Affected by rheumatoid arthritis and Sjögren’s

PHYSICAL FINDINGS & CLINICAL PRESENTATION See Table 1-18.

ETIOLOGY See Table 1-18.

DIAGNOSIS DIFFERENTIAL DIAGNOSIS Logical approach to loss of vision: generally helpful to categorize vision loss as sudden vs. gradual, unilateral vs. bilateral, and painless vs. painful. Sudden—Unilateral—Painless loss of vision • Vitreous hemorrhage—may resolve spontaneously, but in people with DM, may need emergency photocoagulation • Retinal detachment—requires urgent surgical repair • Age-related macular degeneration/ choroidal neovascular membrane— urgent angiography to detect lesions amenable to repair • Retinal vein occlusion—urgent retinal photocoagulation • Amaurosis fugax and central retinal artery occlusion—urgent neurovascular workup • Anterior ischemic optic neuropathy— also requires urgent neurovascular workup, but may have nonarteritic causes

DISEASES AND DISORDERS

• Vision loss in the elderly incorporates the spectrum from near-normal vision to mild deficits to low vision, profound visual loss, and blindness. • Moderate and severe vision loss can have a profound impact on the ability to perform instrumental activities of daily living such as reading, driving, check-writing, and taking medications. • Vision loss can result in an inability to live independently and to socialize. • Vision loss increases the risk of depression, functional impairment, and mortality.

Sudden—Unilateral—Painful loss of vision • Associated with red eye  Corneal ulcers and infections— emergency ophthalmologic referral for cultures and intensive antibiotic treatment  Corneal abrasions  Acute angle-closure glaucoma— emergency laser iridectomy to relieve blockage • Associated with photophobia  Uveitis—may be idiopathic, but associated with sarcoid, collagen vascular disease, syphilis, and TB; refer to ophthalmology for slit lamp exam and topical steroids • Traumatic hyphema—refer to ophthalmology and minimize intraocular pressure and risk of recurrent bleeding • Pain and tenderness over scalp, elevated ESR—temporal arteritis  Requires urgent treatment with systemic corticosteroids • Pain on eye movement—optic neuritis  Associated with multiple sclerosis  May respond to systemic corticosteroids • Orbital cellulitis  Emergency referral to ophthalmologist and otorhinolaryngologist  Cultures, CT scanning, IV antibiotics Sudden—Bilateral—Painless loss of vision • Uncontrolled diabetes • Medications, especially anticholinergics and corticosteroids Sudden—Bilateral—Painful loss of vision • Trauma, foreign bodies, chemical spills, exposure to UV radiation • Corneal infections, iritis, acute glaucoma—all rarely bilateral • Temporal arteritis Gradual—Unilateral—Painless loss of vision • Cataracts—require routine, nonurgent ophthalmologic follow-up • Age-related macular degeneration— routine ophthalmologic follow-up • Lesions in the orbit or intracranial space—visual field defects and optic atrophy with lesions compressing the optic nerve Gradual—Unilateral—Painful loss of vision • Inflammatory or neoplastic process of the cornea or retrobulbar space, including orbital granulomas and optic neuromas—require ophthalmologic referral and CT or MRI Gradual—Bilateral—Painless loss of vision • Cataracts develop with age • Age-related macular degeneration • Drug toxicity, such as hydroxychloroquine or ethambutol • Compressive lesions at the optic chiasm

Section I

DEFINITION

Cornea • Yellows with age, loses transparency, becomes more astigmatic • Fuch’s dystrophy is an autosomal dominant disease found after the fifth decade causing severe pain and loss in corneal transparency • Affected by infections, dry eyes, and neoplasms Pupil • Aperture decreases by 2.5 mm between age 20 and 80, resulting in less ambient light reaching the photosensitive layer Lens • Yellowing of the lens with reduced amount of light reaching the retina • Loss of accommodative amplitude (presbyopia) begins around age 40, with impairment in the fifth to sixth decades • Cataracts Vitreous • Transparent but may contract, liquefy, or detach • Vitreous hemorrhage is a complication of diabetic retinopathy that causes clouded vision Retina • Affected by pathologic changes in macular degeneration, diabetic retinopathy, retinal detachment, artery and vein occlusions, or hemorrhage

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Vision Loss TABLE 1-18

Causes of Vision Loss

Disease

Symptoms

Exam Findings and Diagnostic Tests

Treatment

Amaurosis fugax

• Rapid unilateral vision loss • Curtain descending over vision • Sudden loss of a portion of visual field

• Treat hypertensive crisis • Treat ischemic disease • Treat hypercoagulable state

Anterior ischemic optic neuropathy (AION)

• Sudden unilateral vision loss • May become progressive

Retinal detachment

• • • •

• Ophthalmoscopy: retinal edema (appears whitened); milky retina with a cherry red fovea • Evaluate for ischemic disease, including neuroimaging, carotid Doppler ultrasonography, or echocardiogram • May need to evaluate for hypercoagulable state • Ophthalmoscopy: optic disc swollen; nerve fiber layer splinter hemorrhages • Check ESR and CRP (association with temporal arteritis) • Ophthalmoscopy: elevated, folded retinal tissue • Decreased visual acuity

• Ophthalmoscopy: engorged, phlebitic veins; retinal hemorrhages • May need to evaluate for hypercoagulable state • Optic disc swelling • History of drug exposure: carbon monoxide, ethylene glycol, methanol, amiodarone, ciprofloxacin, digitalis, thallium, isoniazid, and others • Ophthalmoscopy • Ophthalmoscopy: drusen; degeneration of the retinal pigmented epithelium • Fluorescein angiography showing choroidal neovascular lesions

• Treat underlying hypertension and diabetes • Treat hypercoagulable state • Aspirin • Discontinue offending agent

• Ophthalmoscopy: optic nerve cupping (increased cup to disk ratio); nasalization of retinal vessels • Loss of visual fields • Visual field testing showing loss of fields • Tonometry showing increased intraocular pressure • Visual acuity testing showing loss of accommodative amplitude

• Eyedrops, including beta blockers, cholinergic agonists, prostaglandin analogs, carbonic anhydrase inhibitors • Surgery, including iridotomy or filtration surgery

Retinal vein occlusion

Floaters Flashing lights Peripheral scotoma Afferent pupil defect and decreased acuity if fovea involved • Similar to amaurosis fugax, but more prolonged

Toxic optic neuropathy

• Acute bilateral vision loss • Central scotoma

Age-related macular degeneration

• Blurring of central vision (peripheral intact) • Central scotoma • Glare sensitivity • Decreased color perception • Hallucinations • Early  asymptomatic • Headache or blurred vision after exercise • Haloes or cloudy vision in low illumination • Blurred vision • Loss of peripheral vision or tunnel vision • Loss of ability to focus on close objects

Glaucoma

Presbyopia

Cataract

Diabetic retinopathy

• • • • • • • • • • • •

Blurred vision Light sensitivity Small local opacity Double vision Worsening myopia Reduced contrast sensitivity May be asymptomatic, especially nonproliferative type Floaters Blurred vision Decreased color perception Decreased night vision Symptoms worse in the morning

• Reduced visual acuity • Loss of red reflex • Focal or diffuse opacity of lens

• Ophthalmoscopy: retinal changes, including hemorrhages and exudates; macular edema; vitreous hemorrhage; vascular changes including looping

• High-dose glucocorticoids • May need temporal artery biopsy

• Laser surgery

• Vitamin and mineral supplements may reduce risk • Laser photocoagulation • Photodynamic therapy

• • • • • • •

Prescriptive lenses Bifocal contact lenses Corrective corneal surgery Lens implantation Absorptive lenses that filter glare Lens prescription adjustment Intraocular lens implantation surgery

• Control blood sugar • Treat hypertension and hypercholesterolemia • Focal laser photocoagulation • Scatter laser photocoagulation • Vitrectomy

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Vision Loss

TREATMENT

See Table 1-18.

NONPHARMACOLOGIC THERAPY See individual topics for condition-specific treatment. • Vision rehabilitation  Useful for patients with macular degeneration, cataracts, glaucoma, diabetic retinopathy, or visual field loss.  Patients with low visual acuity (20/50), central or peripheral vision loss, reduced contrast sensitivity, glare, and light/dark adaptation difficulties may benefit.  Assessment of need for low-vision devices, reading ability, impairment in activities of daily living, ability to travel. • Low-vision devices  Magnifiers and telescopic systems for macular degeneration and visual field losses  Absorptive and tinted lenses for glare  Visual field expansion devices, including mirrors and prisms  Illumination control for light/dark adaptation  Use of increased bright color or color contrast

REFERRAL See individual topics for condition-specific referral. • Ophthalmologist—emergent visit with acute symptoms • Otolaryngologist—for neoplastic or infectious concerns

PEARLS & CONSIDERATIONS Don’t forget the impact of vision loss on the elderly, including increased depression, loss of functional status, and increased mortality.

PREVENTION • Antioxidants for macular degeneration  Lutein alone or with antioxidants was helpful in the Veterans’ Lutein Antioxidant Supplement Trial.  The Age-Related Eye Diseases Study showed benefit of antioxidants in patients with advanced macular degeneration, but other studies have not shown benefit of antioxidants for primary prevention. • Diabetic retinopathy  Better glycemic control: Most of the preventive benefit is achieved by reducing a high HbA1c to 9%, and little is achieved by reducing from 9% to 7%.  Control hypertension  Control hyperlipidemia • Smoking cessation to reduce the risk of macular degeneration, cataracts, and diabetic retinopathy • Avoid ultraviolet light damage

PATIENT/FAMILY EDUCATION • General vision and information and referral for vision disorders: http://www.visionchannel.net • Lighthouse for the Blind: http://www.lighthouse.org • American Council for the Blind: http://www.acb.org • Macular degeneration: www.mdsupport.org • Glaucoma Service Foundation To Prevent Blindness: http://www.wills-glaucoma.org/index.htm

CROSS REFERENCES Please refer to the following topics for more information about specific diseases causing vision loss: Cataracts Glaucoma, Chronic Open-Angle Glaucoma, Primary Closed-Angle Macular Degeneration Red Eye, Acute (algorithm)

SUGGESTED READINGS Harvey PT: Common eye diseases of elderly people: identifying and treating causes of vision loss, Gerontology 49:1-11, 2003. Quillen DA: Common causes of vision loss in elderly patients, Amer Fam Phys 60:99-108, 1999. Reuben DB et al: The prognostic value of sensory impairment in older persons, J Am Geriatr Soc 47:930-935, 1999. Rosenthal BP: Screening and treatment of agerelated and pathologic vision changes, Geriatrics 58:27-31, 2001. Shingleton BJ, O’Donoghue MW: Blurred vision, N Engl J Med 343:556-562, 2000. AUTHORS: HOLLY M. HOLMES, M.D., and JOSEPH W. SHEGA, M.D.

DISEASES AND DISORDERS

WORKUP, LABORATORY TESTS, IMAGING STUDIES

• Low-vision evaluation  For patients with vision not corrected by glasses, lenses, medical treatments, or surgery  Specialized tests of function, including contrast sensitivity, Amsler grid (for macular degeneration), visual field testing, color vision testing, and photostress tests • Lighthouse for the Blind: local chapters providing support, services, products, referrals, and rehabilitation services for visually impaired persons • Local Department on Aging chapters: information about senior centers that provide services and rehabilitation for vision loss

Section I

Gradual—Bilateral—Painful loss of vision • Chronic inflammatory process, associated with collagen vascular disease or sarcoidosis

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Vulvar Cancer BASIC INFORMATION DEFINITION Vulvar cancer is an abnormal cell proliferation arising on the vulva and exhibiting malignant potential. The majority are of squamous cell origin; however, other types include adenocarcinoma, basal cell carcinoma, sarcoma, and melanoma (Fig. 1-52).

SYNONYMS Squamous cell carcinoma of the vulva (90%) Basal cell carcinoma of the vulva Adenocarcinoma of the vulva Melanoma of the vulva Bartholin gland carcinoma Verrucous carcinoma of the vulva Vulvar sarcoma ICD-9CM CODES 184.4 Vulvar neoplasm

EPIDEMIOLOGY & DEMOGRAPHICS PREVALENCE: Vulvar cancer is uncommon. It comprises 4% of malignancies of the female genital tract. It is the fourth most common gynecologic malignancy. INCIDENCE: 1.8 cases/100,000 persons

MEAN AGE AT DIAGNOSIS: Predominantly a disease of menopause. Mean age at diagnosis is 65 yr.

DIAGNOSIS

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• • • • •

• Vulvar pruritus or pain is present. • May produce a malodor or discharge or present as bleeding. • Raised lesion, may have fleshy, ulcerated, leukoplakic, or warty appearance; may have multifocal lesions. • Lesions are usually located on labia majora, but may be seen on labia minora, clitoris, and perineum. • The lymph nodes of groin may be palpable.

ETIOLOGY • The exact etiology is unknown. • Vulvar intraepithelial neoplasia has been reported in 20%-30% of invasive squamous cell carcinoma of the vulva, but the malignant potential is unknown. • Human papillomavirus is found in 30%-50% of vulvar carcinoma, but its exact role is unclear. • Chronic pruritus, wetness, industrial wastes, arsenicals, hygienic agents, and vulvar dystrophies have been implicated as causative agents.

A

DIFFERENTIAL DIAGNOSIS Lymphogranuloma inguinale Tuberculosis Vulvar dystrophies Vulvar atrophy Paget’s disease

WORKUP • Diagnosis is made histologically by biopsy • Thorough examination of the lesion and assessment of spread • Possible colposcopy of adjacent areas • Cytologic smear of vagina and cervix • Cystoscopy and proctosigmoidoscopy may be necessary

IMAGING STUDIES • Chest radiography • CT scan and MRI for assessing local tumor spread

TREATMENT NONPHARMACOLOGIC THERAPY • Treatment is individualized depending on the stage of the tumor. • Stage I tumors with 1 mm stromal invasion are treated with complete local excision without groin node dissection. • Stage I tumors with 1 mm stromal invasion are treated with complete local excision with groin node dissection. • Stage II tumors require radical vulvectomy with bilateral groin node dissection. • Advanced-stage disease may require the addition of radiation and chemotherapy to the surgical regimen. • Section III describes a treatment algorithm for management of vulvar cancer.

DISPOSITION Five-year survival ranges from 90% for stage I to 15% for stage IV.

REFERRAL Vulvar cancer should be managed by a gynecologic oncologist and radiation oncologist.

B

SUGGESTED READINGS Canavan TP, Cohen D: Vulvar cancer, Am Fam Physician 66(7):1269, 2002. Coleman RL, Santoso JT: Vulvar carcinoma, Curr Treat Opt Oncol 1(2):177, 2000. Grandys EC Jr, Aroris JV: Innovations in the management of vulvar carcinoma, Curr Opin Obstet Gynecol 12(1):15, 2000.

FIGURE 1-52 A, Basal cell carcinoma of the vulva. B, Ulcerative squamous cell carcinoma of the vulva. (From Symonds EM, Macpherson MBA: Color atlas of obstetrics and gynecology, St Louis, 1994, Mosby.)

AUTHOR: GIL FARKASH, M.D.

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Vulvovaginitis, Estrogen-Deficient EPIDEMIOLOGY & DEMOGRAPHICS

DEFINITION

• Seen most often in postmenopausal women

SYNONYMS Atrophic vaginitis ICD-9CM CODES 616.10 Vulvovaginitis

PHYSICAL FINDINGS & CLINICAL PRESENTATION • Thinning of pubic hair, labia minora and majora • Decreased secretions from the vestibular glands, with vaginal dryness • Regression of subcutaneous fat • Vulvar and vaginal itching • Dyspareunia • Dysuria and urinary frequency • Vaginal spotting

ETIOLOGY Estrogen deficiency

DIAGNOSIS

CHRONIC Rx

DIFFERENTIAL DIAGNOSIS

See “Acute General Rx.” May discontinue vaginal estrogen cream once symptoms alleviate.

• • • • • •

FIGURE 1-53 Advanced postmenopausal atrophy of the vulva in a 72year-old woman. (From Symonds EM, Macpherson MBA: Color atlas of obstetrics and gynecology, St Louis, 1994, Mosby.)

Infectious vulvovaginitis Squamous cell hyperplasia Lichen sclerosus Vulva malignancy Vaginal malignancy Cervical and endometrial malignancy

DISPOSITION The symptoms should be improved with the therapy. Caution for vaginal bleeding if uterus present

WORKUP

REFERRAL

• • • •

To obstetrician/gynecologist if vaginal bleeding

Pelvic examination Speculum examination Pap smear Possible endometrial biopsy if bleeding

SUGGESTED READING

TREATMENT

Bornstein J et al: The classic approach to diagnosis of vulvovaginitis: a critical analysis, Infect Dis Obstet Gynecol 9(2):105, 2001.

ACUTE GENERAL Rx

AUTHOR: JULIE ANNE SZUMIGALA, M.D.

• Premarin 0.625 mg PO qd • Estraderm patch 0.05 mg  2 per week

DISEASES AND DISORDERS

Estrogen-deficient vulvovaginitis is the irritation and/or inflammation of the vulva and vagina because of progressive thinning and atrophic changes secondary to estrogen deficiency (Fig. 1-53).

• If uterus present: 1. Estrogen  2.5 mg PO Provera qd or 2. Estrogen  10 mg PO Provera  10 days each mo • Systemic use of HRT or ERT is controversial at this time. • Conjugated estrogen vaginal cream intravaginally. Estradiol vaginal cream 0.01% 2 to 4 g/day  2 wk then 1 to 2 g/day  2 wk then 1 to 2 g  3 days/wk • Vagifen (estradial vaginal tablets) 25 mg inserted intravaginally daily for 2 wk then twice weekly. May take up to 12 wk to feel the full benefits of the medication. • Conjugated estrogen vaginal cream: 24 g qd (3 wk on, 1 wk off) for 3-5 mo.

Section I

BASIC INFORMATION

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Wounds BASIC INFORMATION

PHYSICAL FINDINGS & CLINICAL PRESENTATION

DEFINITION

Pressure ulcers usually occur over bony prominences and are graded or staged to classify the degree of tissue damage observed. Stage I: nonblanchable erythema of intact skin Stage II: partial-thickness skin loss involving the epidermis, dermis, or both Stage III: full-thickness skin loss involving damage or necrosis of subcutaneous tissue that may extend down to, but not through, underlying fascia or muscle Stage IV: full-thickness skin loss with extensive destruction and tissue damage to muscle, bone, or supporting structures

Pressure ulcers: localized areas of tissue necrosis that develop when soft tissue is compressed between a bony prominence and an external surface for a prolonged period. Arterial ulcers: areas of arteriosclerosis that lead to insufficient oxygenation of the skin and underlying tissues. Diabetic ulcers: a chronic ulcer in a diabetic patient due to extrinsic factors (e.g., trauma, injury) or intrinsic factors (e.g., neuropathy, microvascular disease). Venous ulcers: a discontinuity of the epidermis occurring as a result of venous hypertension and venous insufficiency.

SYNONYMS Decubitus ulcers Bedsores Pressure sores Dermal ulcers ICD-9CM CODES 707.0 Decubitus ulcers 440.23 Arterial peripheral vascular disease with ulceration 459.81 Venous insufficiency 707.10-707.9 codes specify ulcer sites

EPIDEMIOLOGY & DEMOGRAPHICS • The incidence of pressure ulcers in acute care facilities has ranged from 2.7% to 29.5%. Prevalence has varied from 3.5% to 29.5%. High-risk populations include quadriplegic patients (60% prevalence), elderly patients admitted for femoral fracture (66% incidence), and critical care patients (33% incidence). • Risk factors for pressure ulcers:  Altered sensation or response to discomfort  Altered mobility  Significant changes in weight  Bowel or bladder incontinence • Risk factors for arterial ulcers:  Arterial insufficiency  Tobacco abuse • Risk factors for diabetic ulcers:  Diabetic neuropathy  Structural foot deformity  Peripheral arterial occlusive disease • Risk factors for venous ulcers:  Venous insufficiency  History of leg injury, obesity, phlebitis, varicose vein surgery  History of prolonged sitting or standing

ETIOLOGY • Prolonged unrelieved pressure often associated with impaired or restricted mobility • Friction or shearing forces on skin • See Definition • See Risk Factors

DIAGNOSIS

• Assess all at-risk patients at the time of admission to health care facilities and at regular intervals thereafter. • Identify all individual risk factors (decreased mental status, moisture, incontinence, nutritional deficits) to direct specific preventive treatments. Modify care according to the individual factors. • All ulcers should have a description of the ulcer (i.e., stage, location, size); the wound bed (i.e., epithelialization, granulation tissue, necrotic tissue, eschar); the presence of any exudates, which includes type and amount; the wound edges (i.e., undermining, sinus tracts, tunneling, fistulas); signs of infection; and pain.

LABORATORY TESTS • CBC with differential • Sed rate • Albumin

IMAGING STUDIES X-ray

TREATMENT

DIFFERENTIAL DIAGNOSIS

NONPHARMACOLOGIC THERAPY

Diabetic ulcers (DU) Venous stasis ulcers (VU) Arterial ulcers (AU) See Table 1-19.

Special mattresses/beds (e.g., low air loss mattress) may help reduce skin pressure. They can be used preventively or as part of a treatment program.

WORKUP

CHRONIC Rx

Many pressure ulcers are preventable, with a comprehensive risk assessment in place. Risk assessment: • Consider all bed- or chair-bound persons, or those whose ability to reposition is impaired, to be at risk for pressure ulcers. • Select and use a method of risk assessment, such as the Norton Scale or Braden Scale, that ensures systematic evaluation of individual risk factors.

Pressure ulcers: • Managing tissue loads. Reduce skin pressure through repositioning and pressure-reducing devices. Positioning devices should be used to raise the ulcer off the support surface. Prevent shear injury by maintaining the head of the bed at the lowest level of elevation and for the shortest period that is consistent with medical conditions and other restrictions.

TABLE 1-19

Differential Diagnosis of Ulcers

PU—pressure ulcers DU—diabetic ulcers VU—venous statis ulcers AU—arterial ulcers

Location

Cause

Appearance

Bony prominences Pressure points (callus) Gaiter area

Pressure Repetitive trauma; neuropathy Venous stasis

Crater Well-marked borders Irregular

Distal points

Vascular insufficiency

Gangrene

From Thomas D: Wound care: prevention, management, and nursing care, American Medical Director’s Association Annual Symposium, Phoenix, Arizona, 2004. Copyright 2004.

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Wounds COMPLEMENTARY & ALTERNATIVE MEDICINE • Nutritional and vitamin supplementation have a strength of evidence C, per ACHPR. • Encourage dietary intake and supplementation if an individual with a pressure ulcer is malnourished. • A daily high-potency vitamin/mineral is recommended for all patients suspected of having deficiencies.

DISPOSITION When systematic risk assessments are done and preventive measures are followed, many pressure ulcers can be prevented. Most ulcers heal when appropriate management strategies are followed.

REFERRAL Surgical referral when clean Stage III or IV pressure ulcers do not respond to optimal patient care.

SUGGESTED READINGS National Pressure Ulcer Advisory Panel: Statement on pressure ulcer prevention: http://www.npuap.org/positn1.html Thomas D: Wound care: prevention, management, and nursing care, American Medical Director’s Association Annual Symposium, Phoenix, Arizona, 2004. Treatment of pressure ulcers, Clinical Practice Guidelines Number 15, AHCPR Publication No. 95-0652, Rockville, Md, 1994, US Department of Health and Human Services, Public Health Service, Agency for Health Care Policy and Research. AUTHOR: LAURA TRICE, M.D.

DISEASES AND DISORDERS

flaps. Quality of life, patient preferences, treatment goals, risk of recurrence, and anticipated rehabilitative outcomes should be considered. • Education and quality improvement. Institutions and health care agencies are responsible for developing and implementing pressure ulcer prevention and treatment programs for patients, families, and caregivers. In addition, a quality improvement program should be established. Arterial ulcers: • Restore adequate vascular supply whenever possible. • Consider aspirin 325 mg qd, cilostazol (Pletal) 100 mg bid, or pentoxifylline (Trental) 400 mg tid. • Manage underlying medical issues, e.g., hypertension, diabetes. • Maintain moderate level of exercise. • Smoking cessation. • Avoid caffeine, cold temperatures, and constrictive garments. • Keep wounds clean. • Use nonocclusive moist wound dressings. • Manage local infection. • Treatment failure may lead to amputation. Diabetic ulcers: • Offload mechanical stress. • Sharp wound debridement. • Revascularization. • Moist wound dressing. • Manage local infection. • Treatment failure may lead to amputation. Venous ulcers: • Use a compression bandage system. • Manage local infection. • Moist wound dressing. • Use bioengineered skin appropriately.

Section I

• Ulcer care. Any necrotic tissue observed during initial or subsequent assessments should be debrided. The clinician should select the debridement method (sharp, mechanical, enzymatic, or autolytic) most appropriate to the patient’s condition and goals. Ulcer wounds should be cleansed (preferably with normal saline) at each dressing change, using a minimum amount of mechanical force. Select a dressing that will keep the ulcer bed continuously moist while allowing the surrounding intact skin to remain dry. Consider whirlpool therapy for cleansing pressure ulcers that contain thick exudates, slough, or necrotic tissue. • Managing bacterial colonization and infection. All Stage II, III, and IV pressure ulcers are colonized with bacteria. Effective cleansing and debridement will minimize colonization. More frequent cleansing and debridement may be needed if purulence or a foul odor develops. A topical antibiotic should be considered if a clean ulcer continues to have exudates despite optimal care for 2 to 4 weeks. Topical antiseptics should not be used. Swab cultures have no diagnostic value; however, if the ulcer does not respond to topical antibiotic therapy, quantitative bacterial cultures, preferably by means of tissue biopsy, should be obtained to evaluate for osteomyelitis. Systemic antibiotics should be given to patients with bacteremia, sepsis, advancing cellulitis, or osteomyelitis. • Operative repair. Operative procedures should be considered for clean Stage III or IV ulcers that do not respond to optimal care and include direct closure, skin grafting, skin flaps, musculocutaneous flaps, and free

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Abdominal Distention ABDOMINAL DISTENTION ICD-9CM # 787.3

NONMECHANICAL OBSTRUCTION

MECHANICAL OBSTRUCTION

ABDOMINAL PAIN, DIFFUSE ICD-9CM # 789.67 Early appendicitis. Aortic aneurysm. Gastroenteritis. Intestinal obstruction. Diverticulitis. Peritonitis. Mesenteric insufficiency or infarction. Pancreatitis. Inflammatory bowel disease. Irritable bowel. Mesenteric adenitis. Metabolic: toxins, lead poisoning, uremia, drug overdose, diabetic ketoacidosis (DKA), heavy metal poisoning. Sickle cell crisis. Pneumonia (rare). Trauma. Urinary tract infection. Other: acute intermittent porphyria, tabes dorsalis, periarteritis nodosa, Henoch-Schönlein purpura, adrenal insufficiency.

ABDOMINAL PAIN, EPIGASTRIC ICD-9CM # 789.66 Gastric: peptic ulcer disease (PUD), gastric outlet obstruction, gastric ulcer. Duodenal: PUD, duodenitis. Biliary: cholecystitis, cholangitis.

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Hepatic: hepatitis. Pancreatic: pancreatitis. Intestinal: high small bowel obstruction, early appendicitis. Cardiac: angina, MI, pericarditis. Pulmonary: pneumonia, pleurisy, pneumothorax. Subphrenic abscess. Vascular: dissecting aneurysm, mesenteric ischemia.

ABDOMINAL PAIN, LEFT LOWER QUADRANT ICD-9CM # 789.64 Intestinal: diverticulitis, intestinal obstruction, perforated ulcer, inflammatory bowel disease, perforated descending colon, inguinal hernia, neoplasm, appendicitis. Reproductive: ovarian cyst, torsion of ovarian cyst, tuboovarian abscess. Renal: renal or ureteral calculi, pyelonephritis, neoplasm. Vascular: leaking aortic aneurysm. Psoas abscess. Trauma.

ABDOMINAL PAIN, LEFT UPPER QUADRANT ICD-9CM # 789.32 Gastric: PUD, gastritis, pyloric stenosis, hiatal hernia. Pancreatic: pancreatitis, neoplasm, stone in pancreatic duct or ampulla. Cardiac: MI, angina pectoris. Splenic: splenomegaly, ruptured spleen, splenic abscess, splenic infarction. Renal: calculi, pyelonephritis, neoplasm. Pulmonary: pneumonia, empyema, pulmonary infarction. Vascular: ruptured aortic aneurysm. Cutaneous: herpes zoster. Trauma. Intestinal: high fecal impaction, perforated colon, diverticulitis.

ABDOMINAL PAIN, PERIUMBILICAL ICD-9CM # 789.65 Intestinal: small bowel obstruction or gangrene, early appendicitis. Vascular: mesenteric thrombosis, dissecting aortic aneurysm. Pancreatic: pancreatitis. Metabolic: uremia, DKA. Trauma.

ABDOMINAL PAIN, RIGHT LOWER QUADRANT ICD-9CM # 789.63 Intestinal: acute appendicitis, regional enteritis, incarcerated hernia, cecal diverticulitis, intestinal obstruction, perforated ulcer, perforated cecum, Meckel’s diverticulitis. Reproductive: ovarian cyst, torsion of ovarian cyst. Renal: renal and ureteral calculi, neoplasms, pyelonephritis. Vascular: leaking aortic aneurysm. Psoas abscess. Trauma. Cholecystitis.

ABDOMINAL PAIN, RIGHT UPPER QUADRANT ICD-9CM # 789.61 Biliary: calculi, infection, inflammation, neoplasm. Hepatic: hepatitis, abscess, hepatic congestion, neoplasm, trauma.

DIFFERENTIAL DIAGNOSIS

Neoplasm (intraluminal, extraluminal). Adhesions, endometriosis. Infection (intraabdominal abscess, diverticulitis). Gallstones. Foreign body, bezoars. Hernias. Volvulus. Stenosis at surgical anastomosis, radiation stenosis. Fecaliths. Inflammatory bowel disease. Gastric outlet obstruction. Hematoma. Other: parasites, superior mesenteric artery (SMA) syndrome, pneumatosis intestinalis, annular pancreas, Hirschsprung’s disease, intussusception, meconium.

Abdominal Pain, Right Upper Quadrant

Section II

Excessive intraluminal gas. Intraabdominal infection. Trauma. Retroperitoneal irritation (renal colic, neoplasms, infections, hemorrhage). Vascular insufficiency (thrombosis, embolism). Mechanical ventilation. Extraabdominal infection (sepsis, pneumonia, empyema, osteomyelitis of spine). Metabolic/toxic abnormalities (hypokalemia, uremia, lead poisoning). Chemical irritation (perforated ulcer, bile, pancreatitis). Peritoneal inflammation. Severe pain, pain medications.

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Abdominal Pain, Suprapubic Gastric: PUD, pyloric stenosis, neoplasm, alcoholic gastritis, hiatal hernia. Pancreatic: pancreatitis, neoplasm, stone in pancreatic duct or ampulla. Renal: calculi, infection, inflammation, neoplasm, rupture of kidney. Pulmonary: pneumonia, pulmonary infarction, right-sided pleurisy. Intestinal: retrocecal appendicitis, intestinal obstruction, high fecal impaction, diverticulitis. Cardiac: myocardial ischemia (particularly involving the inferior wall), pericarditis. Cutaneous: herpes zoster. Trauma. Fitz-Hugh-Curtis syndrome (perihepatitis).

ABDOMINAL PAIN, SUPRAPUBIC ICD-9CM # 789.85 Intestinal: colon obstruction or gangrene, diverticulitis, appendicitis. Reproductive system: torsion of ovarian cyst. Cystitis, rupture of urinary bladder.

ACHES AND PAINS, DIFFUSE13 ICD-9CM # 719.49 Postviral arthralgias/myalgias. Bilateral soft tissue rheumatism. Overuse syndromes. Fibrositis. Hypothyroidism. Metabolic bone disease. Paraneoplastic syndrome. Myopathy (polymyositis, dermatomyositis). RA. Sjögren’s syndrome. Polymyalgia rheumatica. Hypermobility. Benign arthralgias/myalgias. Chronic fatigue syndrome. Hypophosphatemia.

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Adrenal Masses ACIDOSIS RESPIRATORY ICD-9CM # 276.2 Pulmonary disease (COPD, severe pneumonia, pulmonary edema, interstitial fibrosis). Airway obstruction (foreign body, severe bronchospasm, laryngospasm). Thoracic cage disorders (pneumothorax, flail chest, kyphoscoliosis). Defects in muscles of respiration (myasthenia gravis, hypokalemia, muscular dystrophy). Defects in peripheral nervous system (amyotrophic lateral sclerosis, poliomyelitis, Guillain-Barré syndrome, botulism, tetanus, organophosphate poisoning, spinal cord injury). Depression of respiratory center (anesthesia, narcotics, sedatives, vertebral artery embolism or thrombosis, increased intracranial pressure). Failure of mechanical ventilator.

ACUTE SCROTUM ICD-9CM # 608.9 Testicular torsion. Epididymitis. Testicular neoplasm. Orchitis.

ADNEXAL MASS14 ICD-9CM # VARIES WITH SPECIFIC DISORDER Ovary (neoplasm, endometriosis, functional cyst). Fallopian tube (neoplasm, tuboovarian abscess, hydrosalpinx, paratubal cyst). Uterus (fibroid, neoplasm). Retroperitoneum (neoplasm, abdominal wall hematoma or abscess). Urinary tract (pelvic kidney, distended bladder, urachal cyst). Inflammatory bowel disease. GI tract neoplasm. Diverticular disease. Appendicitis. Bowel loop with feces.

ACIDOSIS, METABOLIC

ADRENAL MASSES22

ICD-9CM # 276.2

ICD-9CM # 194.0 ADRENOCORTICAL CARCINOMA 255.8 ADRENAL HYPERPLASIA

METABOLIC ACIDOSIS WITH INCREASED AG (AG ACIDOSIS) Lactic acidosis. Ketoacidosis (diabetes mellitus, alcoholic ketoacidosis). Uremia (chronic renal failure). Ingestion of toxins (paraldehyde, methanol, salicylate, ethylene glycol). High-fat diet (mild acidosis).

METABOLIC ACIDOSIS WITH NORMAL AG (HYPERCHLOREMIC ACIDOSIS) Renal tubular acidosis (including acidosis of aldosterone deficiency). Intestinal loss of HCO3 (diarrhea, pancreatic fistula). Carbonic anhydrase inhibitors (e.g., acetazolamide). Dilutional acidosis (as a result of rapid infusion of bicarbonate-free isotonic saline). Ingestion of exogenous acids (ammonium chloride, methionine, cystine, calcium chloride). Ileostomy. Ureterosigmoidostomy. Drugs: amiloride, triamterene, spironolactone, -blockers.

UNILATERAL ADRENAL MASSES Functional lesions Adrenal adenoma. Adrenal carcinoma. Pheochromocytoma. Primary aldosteronism, adenomatous type. Nonfunctional lesions Incidentaloma of adrenal. Ganglioneuroma. Myelolipoma. Hematoma. Adenolipoma. Metastasis.

BILATERAL ADRENAL MASSES Functional lesions ACTH-dependent Cushing’s syndrome. Congenital adrenal hyperplasia. Pheochromocytoma. Conn’s syndrome, hyperplastic variety.

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Adynamic Ileus Micronodular adrenal disease. Idiopathic bilateral adrenal hypertrophy. Nonfunctional lesions Infection (tuberculosis, fungi). Infiltration (leukemia, lymphoma). Replacement (amyloidosis). Hemorrhage. Bilateral metastases.

ADYNAMIC ILEUS14 ICD-9CM # 560.1

ALKALOSIS, METABOLIC CHLORIDE-RESPONSIVE Vomiting. Nasogastric (NG) suction. Diuretics. Posthypercapnic alkalosis. Stool losses (laxative abuse, cystic fibrosis, villous adenoma). Massive blood transfusion. Exogenous alkali administration.

CHLORIDE-RESISTANT Hyperadrenocorticoid states (Cushing’s syndrome, primary hyperaldosteronism, secondary mineralocorticoidism [licorice, chewing tobacco]). Hypomagnesemia. Hypokalemia. Bartter’s syndrome.

ALKALOSIS, RESPIRATORY ICD-9CM # 276.3 Hypoxemia (pneumonia, pulmonary embolism, atelectasis, high-altitude living). Drugs (salicylates, xanthenes, progesterone, epinephrine, thyroxine, nicotine). Central nervous system (CNS) disorders (tumor, cerebrovascular accident [CVA], trauma, infections). Psychogenic hyperventilation (anxiety, hysteria). Hepatic encephalopathy. Gram-negative sepsis. Hyponatremia. Sudden recovery from metabolic acidosis. Assisted ventilation.

Renal failure. Eosinophilic pneumonia. Bronchiolitis obliterans. Pulmonary alveolar proteinosis.

AMNESIA ICD-9CM # 292.83 DRUG INDUCED 300.12 HYSTERICAL 780.9 RETROGRADE 437.7 TRANSIENT GLOBAL Degenerative diseases (e.g., Alzheimer’s, Huntington’s disease). CVA (especially when involving thalamus, basal forebrain, and hippocampus). Head trauma. Postsurgical (e.g., mammillary body surgery, bilateral temporal lobectomy). Infections (herpes simplex encephalitis, meningitis). Wernicke-Korsakoff syndrome. Cerebral hypoxia. Hypoglycemia. CNS neoplasms. Creutzfeldt-Jakob disease. Medications (e.g., midazolam and other benzodiazepines). Psychosis. Malingering.

ANAL INCONTINENCE14 ICD-9CM # 787.6

TRAUMATIC Nerve injured in surgery. Spinal cord injury. Obstetric trauma. Sphincter injury.

NEUROLOGIC Spinal cord lesions. Dementia. Autonomic neuropathy (e.g., diabetes mellitus). Gynecologic: pudendal nerve stretched during surgery. Hirschsprung’s disease.

MASS EFFECT Carcinoma of anal canal. Carcinoma of rectum. Foreign body. Fecal impaction. Hemorrhoids.

MEDICAL Inflammatory disease. Diarrhea. Laxative abuse.

ANAPHYLAXIS11 ALVEOLAR CONSOLIDATION ICD-9CM # 514 Infection. Neoplasm (bronchoalveolar carcinoma, lymphoma). Aspiration. Trauma. Hemorrhage (Wegener’s Goodpasture, bleeding diathesis). ARDS. CHF.

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ICD-9CM # 995.0

PULMONARY Laryngeal edema. Epiglottitis. Foreign body aspiration. Pulmonary embolus. Asphyxiation. Hyperventilation.

DIFFERENTIAL DIAGNOSIS

ICD-9CM # 276.3

Anaphylaxis

Section II

Abdominal trauma. Infection (retroperitoneal, pelvic, intrathoracic). Laparotomy. Metabolic disease (hypokalemia). Renal colic. Skeletal injury (rib fracture, vertebral fracture). Medications (e.g., narcotics).

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Anemia, Drug-Induced CARDIOVASCULAR Myocardial infarction. Arrhythmia. Hypovolemic shock. Cardiac arrest.

CNS Vasovagal reaction. CVA. Seizure disorder. Drug overdose.

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Anemia, Megaloblastic DRUGS THAT MAY PRODUCE OR PROMOTE BLOOD LOSS Aspirin. Alcohol. Nonsteroidal antiinflammatory agents. Corticosteroids. Anticoagulants.

ANEMIA, LOW RETICULOCYTE COUNT1 ICD-9CM # 285.9

ENDOCRINE

MICROCYTIC ANEMIA (MCV 80)

Hypoglycemia. Pheochromocytoma. Carcinoid syndrome.

Iron deficiency. Thalassemia minor. Sideroblastic anemia. Lead poisoning.

PSYCHIATRIC Vocal cord dysfunction syndrome. Munchausen’s disease. Panic attack/globus hystericus.

OTHER Hereditary angioedema. Cord urticaria. Idiopathic urticaria. Mastocytosis. Serum sickness. Idiopathic capillary leak syndrome. Sulfite exposure. Scombroid poisoning (tuna, blue fish, mackerel).

ANEMIA, DRUG-INDUCED10 ICD-9CM # 283.0

DRUGS THAT MAY INTERFERE WITH RED CELL PRODUCTION BY INDUCING MARROW SUPPRESSION OR APLASIA Alcohol. Antineoplastic drugs. Antithyroid drugs. Antibiotics. Oral hypoglycemic agents. Phenylbutazone. Azidothymidine (AZT).

DRUGS THAT INTERFERE WITH VITAMIN B12, FOLATE, OR IRON ABSORPTION OR UTILIZATION Nitrous oxide. Anticonvulsant drugs. Antineoplastic drugs. Isoniazid, cycloserine A.

DRUGS CAPABLE OF PROMOTING HEMOLYSIS Immune mediated Penicillins. Quinine. Alpha-methyldopa. Procainamide. Mitomycin C. Oxidative stress Antimalarials. Sulfonamide drugs. Nalidixic acid.

MACROCYTIC ANEMIA (MCV 100) Megaloblastic anemias. Folate deficiency. Vitamin B12 deficiency. Drug-induced megaloblastic anemia. Nonmegaloblastic macrocytosis. Liver disease. Hypothyroidism.

NORMOCYTIC ANEMIA (MCV 80-100) Early iron deficiency. Aplastic anemia. Myelophthisic disorders. Endocrinopathies. Anemia of chronic disease. Uremia. Mixed nutritional deficiency.

ANEMIA, MEGALOBLASTIC22 ICD-9CM # 281.0 PERNICIOUS ANEMIA 281.1 B12 DEFICIENCY 281.2 FOLATE DEFICIENCY 281.3 B12 WITH FOLATE DEFICIENCY 281.4 PROTEIN OR AMINO ACID DEFICIENCY 281.8 NUTRITIONAL 281.9 NOS

COBALAMIN (CBL) DEFICIENCY Nutritional Cbl deficiency (insufficient Cbl intake): vegetarians, vegans, breast-fed infants of mothers with pernicious anemia. Abnormal intragastric events (inadequate proteolysis of food Cbl): atrophic gastritis, partial gastrectomy with hypochlorhydria. Loss/atrophy of gastric oxyntic mucosa (deficient IF molecules): total or partial gastrectomy, pernicious anemia (PA), caustic destruction (lye). Abnormal events in small bowel lumen: Inadequate pancreatic protease (R-Cbl not degraded, Cbl not transferred to IF). • Insufficiency of pancreatic protease—pancreatic insufficiency. • Inactivation of pancreatic protease—Zollinger-Ellison syndrome. Usurping of luminal Cbl (inadequate Cbl binding to IF). • By bacteria—stasis syndromes (blind loops, pouches of diverticulosis, strictures, fistulas, anastomoses); impaired

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Aneurysms, Thoracic Aorta

Nutritional causes Decreased dietary intake—poverty and famine (associated with kwashiorkor, marasmus), institutionalized individuals (psychiatric/nursing homes), chronic debilitating disease/goats’ milk (low in folate), special diets (slimming), cultural/ethnic cooking techniques (food folate destroyed) or habits (folaterich foods not consumed). Decreased diet and increased requirements: • Pathologic: intrinsic hematologic disease (autoimmune hemolytic disease), drugs, malaria; hemoglobinopathies (SS, thalassemia), RBC membrane defects (hereditary spherocytosis, paroxysmal nocturnal hemoglobinopathy); abnormal hematopoiesis (leukemia/lymphoma, myelodysplastic syndrome, agnogenic myeloid metaplasia with myelofibrosis); infiltration with malignant disease; dermatologic (psoriasis). Folate malabsorption With normal intestinal mucosa: • Some drugs (controversial). • Congenital folate malabsorption (rare). With mucosal abnormalities—tropical and nontropical sprue, regional enteritis. Defective cellular folate uptake— familial aplastic anemia (rare) Inadequate cellular utilization Folate antagonists (methotrexate). Hereditary enzyme deficiencies involving folate. Drugs (multiple effects on folate metabolism) Alcohol, sulfasalazine, triamterine, pyrimethamine, trimethoprim-sulfamethoxazole, diphenylhydantoin, barbiturates.

MISCELLANEOUS MEGALOBLASTIC ANEMIAS (NOT CAUSED BY CBL OR FOLATE DEFICIENCY) Congenital disorders of DNA synthesis (rare) Orotic aciduria, Lesch-Nyhan syndrome, congenital dyserythropoietic anemia. Acquired disorders of DNA synthesis Thiamine-responsive megaloblastosis (rare). Malignancy—erythroleukemia—refractory sideroblastic anemias— all antineoplastic drugs that inhibit DNA synthesis. Toxic—alcohol.

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ANEURYSMS, THORACIC AORTA ICD-9CM # 441.2 Trauma. Infection. Inflammatory (syphilis, Takayasu’s disease). Collagen vascular disease (rheumatoid arthritis, ankylosing spondylitis). Annuloaortic ectasia (Marfan’s syndrome, Ehlers-Danlos syndrome). Congenital. Coarctation. Cystic medial necrosis.

ARTERIAL OCCLUSION7 ICD-9CM # 444.22 ARTERIAL OCCLUSION, LOWER EXTREMITIES 444.21 ARTERIAL OCCLUSION, UPPER EXTREMITIES Thromboembolism (post-MI, mitral stenosis, rheumatic valve disease, atrial fibrillation, atrial myxoma, marantic endocarditis, bacterial endocarditis, Libman-Sacks endocarditis). Atheroembolism (microemboli composed of cholesterol, calcium, and platelets from proximal atherosclerotic plaques). Arterial thrombosis (endothelial injury, altered arterial blood flow, trauma, severe atherosclerosis, acute vasculitis). Vasospasm. Trauma. Hypercoagulable states. Miscellaneous (irradiation, drugs, infections, necrotizing).

ARTHRITIS, MONOARTICULAR AND OLIGOARTICULAR2 ICD-9CM # 715.3 OSTEOARTHRITIS, LOCALIZED 711.9 INFECTIOUS ARTHRITIS 716.6 MONOARTICULAR ARTHRITIS 5TH DIGIT TO BE ADDED TO THE ABOVE DEPENDING ON SITE OF ARTHRITIS 0. SITE UNSPECIFIED 1. SHOULDER REGION 2. UPPER ARM 3. FOREARM 4. HAND 5. PELVIC REGION AND THIGH 6. LOWER LEG 7. ANKLE AND/OR FOOT 8. OTHER SPECIFIED EXCEPT SPINE Septic arthritis (S. aureus, Neisseria gonorrhea, Meningococci, Streptococci, S. pneumoniae, enteric gram-neg bacilli). Crystalline-induced arthritis (gout, pseudogout, calcium oxalate, hydroxyapatite and other basic calcium/phosphate crystals). Traumatic joint injury. Hemarthrosis. Monoarticular or oligoarticular flare of an inflammatory polyarticular rheumatic disease (RA, psoriatic arthritis, Reiter’s syndrome, SLE).

ARTHRITIS, POLYARTICULAR ICD-9CM # 715.09 GENERALIZED OSTEOARTHRITIS, MULTIPLE SITES 716.89 ARTHRITIS, MULTIPLE SITES RA. SLE, other connective tissue diseases, erythema nodosum, palindromic rheumatism, relapsing polychondritis.

DIFFERENTIAL DIAGNOSIS

FOLATE DEFICIENCY

Arthritis, Polyarticular

Section II

bowel motility (scleroderma, pseudoobstruction), hypogammaglobulinemia. • By Diphyllobothrium latum. Disorders of ileal mucosa/IF receptors (IF-Cbl not bound to IF receptors): Diminished or absent IF receptors—ileal bypass/resection/ fistula. Abnormal mucosal architecture/function—tropical/nontropical sprue, Crohn’s disease, TB ileitis, infiltration by lymphomas, amyloidosis. IF-/post IF-receptor defects—Imerslund-Graesbeck syndrome, TC II deficiency. Drug-induced effects (slow K, biguanides, cholestyramine, colchicine, neomycin, PAS). DISORDERS OF PLASMA CBL TRANSPORT (TC II-CBL NOT DELIVERED TO TC II RECEPTORS) Congenital TC II deficiency, defective binding of TC II-Cbl to TC II receptors (rare). METABOLIC DISORDERS (CBL NOT UTILIZED BY CELL) Inborn enzyme errors (rare). Acquired disorders: (Cbl oxidized to cob[III]alamin)—N2O inhalation.

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Ascites Psoriatic arthritis, ankylosing spondylitis. Sarcoidosis. Lyme arthritis, bacterial endocarditis, Neisseria gonorrhoeae infection, rheumatic fever, Reiter’s disease. Crystal deposition disease. Hypersensitivity to serum or drugs. Hepatitis B, HIV, rubella, mumps. Other: serum sickness, leukemias, lymphomas, enteropathic arthropathy, Whipple’s disease, Behçet’s syndrome, HenochSchönlein purpura, familial Mediterranean fever, hypertrophic pulmonary osteoarthropathy.

ASCITES ICD-9CM # 789.5 ASCITES NOS 197.6 ASCITES, CANCEROUS (MALIGNANT) 457.8 ASCITES, CHYLOUS Hypoalbuminemia: nephrotic syndrome, protein-losing gastroenteropathy, starvation. Cirrhosis. Hepatic congestion: CHF, constrictive pericarditis, tricuspid insufficiency, hepatic vein obstruction (Budd-Chiari syndrome), inferior vena cava or portal vein obstruction. Peritoneal infections: TB and other bacterial infections, fungal diseases, parasites. Neoplasms: primary hepatic neoplasms, metastases to liver or peritoneum, lymphomas, leukemias, myeloid metaplasia. Lymphatic obstruction: mediastinal tumors, trauma to the thoracic duct, filariasis. Ovarian disease: Meigs’ syndrome, struma ovarii. Chronic pancreatitis or pseudocyst: pancreatic ascites. Leakage of bile: bile ascites. Urinary obstruction or trauma: urine ascites. Myxedema. Chylous ascites.

ATAXIA ICD-9CM # 781.3 ATAXIA NOS 303.0 ALCOHOLIC, ACUTE 303.9 ALCOHOLIC, CHRONIC 334.3 CEREBELLAR 331.89 CEREBRAL 334.0 FRIEDREICH’S 300.11 HYSTERICAL Vertebral-basilar artery ischemia. Diabetic neuropathy. Tabes dorsalis. Vitamin B12 deficiency. Multiple sclerosis and other demyelinating diseases. Meningomyelopathy. Cerebellar neoplasms, hemorrhage, abscess, infarct. Nutritional (Wernicke’s encephalopathy). Paraneoplastic syndromes. Parainfectious: Guillain-Barré syndrome. Toxins: phenytoin, alcohol, sedatives, organophosphates. Wilson’s disease (hepatolenticular degeneration). Hypothyroidism. Myopathy. Cerebellar and spinocerebellar degeneration: ataxia/telangiectasia, Friedreich’s ataxia. Frontal lobe lesions: tumors, thrombosis of anterior cerebral artery, hydrocephalus. Labyrinthine destruction: neoplasm, injury, inflammation, compression. Hysteria. AIDS.

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Bleeding, Lower GI BACK PAIN ICD-9CM # 724.5 BACK PAIN (POSTURAL) 724.2 LOW BACK PAIN 307.89 BACK PAIN PSYCHOGENIC 724.8 STIFF BACK 847.9 BACK STRAIN 724.6 BACKACHE, SACROILIAC Trauma: injury to bone, joint, or ligament. Mechanical: obesity, fatigue, scoliosis. Degenerative: osteoarthritis. Infections: osteomyelitis, subarachnoid or spinal abscess, TB, meningitis, basilar pneumonia. Metabolic: osteoporosis, osteomalacia, vertebral compression. Vascular: leaking aortic aneurysm, subarachnoid or spinal hemorrhage/infarction. Neoplastic: myeloma, Hodgkin’s disease, carcinoma of pancreas, metastatic neoplasm from breast, prostate, lung. GI: penetrating ulcer, pancreatitis, cholelithiasis, inflammatory bowel disease. Renal: hydronephrosis, calculus, neoplasm, renal infarction, pyelonephritis. Hematologic: sickle cell crisis, acute hemolysis. Gynecologic: neoplasm of uterus or ovary, dysmenorrhea, salpingitis, uterine prolapse. Inflammatory: ankylosing spondylitis, psoriatic arthritis, Reiter’s syndrome. Lumbosacral strain. Psychogenic: malingering, hysteria, anxiety. Endocrine: adrenal hemorrhage or infarction.

BLEEDING, LOWER GI ICD-9CM # 578.9

(ORIGINATING BELOW THE LIGAMENT OF TREITZ) Small intestine Ischemic bowel disease (mesenteric thrombosis, embolism, vasculitis, trauma). Small bowel neoplasm: leiomyomas, carcinoids. Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Meckel’s diverticulum and other small intestine diverticula. Aortoenteric fistula. Intestinal hemangiomas: blue rubber bleb nevi, intestinal hemangiomas, cutaneous vascular nevi. Hamartomatous polyps: Peutz-Jeghers syndrome (intestinal polyps, mucocutaneous pigmentation). Infections of small bowel: tuberculous enteritis, enteritis necroticans. Volvulus. Intussusception. Lymphoma of small bowel, sarcoma, Kaposi’s sarcoma. Irradiation ileitis. AV malformation of small intestine. Inflammatory bowel disease. Polyarteritis nodosa. Other: pancreatoenteric fistulas, Henoch-Schönlein purpura, Ehlers-Danlos syndrome, systemic lupus erythematosus, amyloidosis, metastatic melanoma. Colon Carcinoma (particularly left colon). Diverticular disease. Inflammatory bowel disease. Ischemic colitis. Colonic polyps.

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Bleeding, Upper GI Vascular abnormalities: angiodysplasia, vascular ectasia. Radiation colitis. Infectious colitis. Uremic colitis. Aortoenteric fistula. Lymphoma of large bowel. Hemorrhoids. Anal fissure. Trauma, foreign body. Solitary rectal/cecal ulcers. Long-distance running.

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Breath Odor METAPHYSIS Parosteal sarcoma. Chondrosarcoma. Fibrosarcoma. Nonossifying fibroma. Giant cell tumor—before fusion of growth plate. Unicameral bone cyst. Aneurysmal bone cyst.

DIAPHYSIS

BLEEDING, UPPER GI

Myeloma. Ewing’s tumor. Reticulum cell sarcoma.

ICD-9CM # 578.9

METADIAPHYSEAL

(ORIGINATING ABOVE THE LIGAMENT OF TREITZ)

ICD-9CM # 369.4 Cataracts. Glaucoma. Diabetic retinopathy. Macular degeneration. Trauma. CVA. Corneal scarring. Temporal arteritis.

BONE LESIONS, PREFERENTIAL SITE OF ORIGIN21 ICD-9CM # 170.0 170.1 170.2 170.3 170.4 170.5 170.6 170.7 170.8 170.9 198.5

SKULL AND FACE MANDIBLE VERTEBRAL COLUMN RIBS, STERNUM, CLAVICLE SCAPULA, LONG BONES UPPER LIMB SHORT BONES AND UPPER LIMB PELVIC BONES, SACRUM COCCYX LONG BONES LOWER LIMB SHORT BONES LOWER LIMB BONE CANCER NOS BONE CANCER, METASTATIC

EPIPHYSIS Chondroblastoma. Giant-cell tumor—after fusion of growth plate. Langerhans’ cell histiocytosis. Clear cell chondrosarcoma. Osteosarcoma.

BRADYCARDIA, SINUS7 ICD-9CM # 427.89 Idiopathic. Degenerative processes (e.g., Lev’s disease, Lenegre’s disease). Medications Beta blockers. Some calcium channel blockers (diltiazem, verapamil). Digoxin (when vagal tone is high). Class I antiarrhythmic agents (e.g., procainamide). Class III antiarrhythmic agents (amiodarone, sotalol). Clonidine. Lithium carbonate. Acute myocardial ischemia and infarction Right or left circumflex coronary artery occlusion or spasm. High vagal tone (e.g., athletes).

BREAST MASS ICD-9CM # 611.72 Fibrocystic breasts. Benign tumors (fibroadenoma, papilloma). Mastitis (acute bacterial mastitis, chronic mastitis). Malignant neoplasm. Fat necrosis. Hematoma. Duct ectasia. Mammary adenosis.

BREATH ODOR20 ICD-9CM # 784.9 HALITOSIS Sweet, fruity: DKA, starvation ketosis. Fishy, stale: uremia (trimethylamines). Ammonia-like: uremia (ammonia). Musty fish, clover: fetor hepaticus (hepatic failure). Foul, feculent: intestinal obstruction/diverticulum. Foul, putrid: nasal/sinus pathology (infection, foreign body, cancer), respiratory infections (empyema, lung abscess, bronchiectasis). Halitosis: tonsillitis, gingivitis, respiratory infections, Vincent’s angina, gastroesophageal reflux, achalasia. Cinnamon: pulmonary TB.

DIFFERENTIAL DIAGNOSIS

BLINDNESS

Fibrosarcoma. Fibrous dysplasia. Enchondroma. Osteoid osteoma. Chondromyofibroma.

Section II

Oral or pharyngeal lesions: swallowed blood from nose or oropharynx. Swallowed hemoptysis Esophageal: varices, ulceration, esophagitis, Mallory-Weiss tear, carcinoma, trauma. Gastric: peptic ulcer (including Cushing and Curling’s ulcers), gastritis, angiodysplasia, gastric neoplasms, hiatal hernia, gastric diverticulum, pseudoxanthoma elasticum, RenduOsler-Weber syndrome. Duodenal: peptic ulcer, duodenitis, angiodysplasia, aortoduodenal fistula, duodenal diverticulum, duodenal tumors, carcinoma of ampulla of Vater, parasites (e.g., hookworm), Crohn’s disease. Biliary: hematobilia (e.g., penetrating injury to liver, hepatobiliary malignancy, endoscopic papillotomy).

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Bullous Diseases BULLOUS DISEASES ICD-9CM # 694.9 694.5 694.4 694.4

BULLOUS DERMATOSES BULLOUS PEMPHIGOID PEMPHIGUS VULGARIS PEMPHIGUS FOLIACEUS

Bullous pemphigoid. Pemphigus vulgaris. Pemphigus foliaceus. Paraneoplastic pemphigus. Cicatricial pemphigoid. Erythema multiforme. Dermatitis herpetiformis. Herpes. Impetigo. Erosive lichen planus. Linear IgA bullous dermatosis. Epidermolysis bullosa acquisita.

CALCIFICATION ON CHEST X-RAY ICD-9CM # 722.92 Lung neoplasm (primary or metastatic). Silicosis. Idiopathic pulmonary fibrosis. Tuberculosis. Histoplasmosis. Disseminated varicella infection. Mitral stenosis (end-stage). Secondary hyperparathyroidism.

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Cardiac Murmurs High-output states Severe anemia. Thyrotoxicosis. Bradycardia Severe sinus bradycardia. Complete heart block.

LEFT ATRIUM LV failure of any cause. Mitral valve disease. Myxoma.

RIGHT VENTRICLE Chronic volume overload. Tricuspid or pulmonic regurgitation. Left-to-right shunt (ASD). Decompensated pressure overload: Pulmonic stenosis. Pulmonary artery hypertension: Primary. Secondary (PE, COPD). Pulmonary venoocclusive disease.

RIGHT ATRIUM RV failure of any cause. Tricuspid valve disease. Myxoma. Ebstein’s anomaly.

MULTICHAMBER ENLARGEMENT

CARDIAC ARREST, NONTRAUMATIC14

Hypertrophic cardiomyopathy. Acromegaly. Severe obesity.

ICD-9CM # 427.5 CARDIAC ARREST NOS

PERICARDIAL DISEASE

Cardiac (coronary artery disease, cardiomyopathies, structural abnormalities, valve dysfunction, arrhythmias). Respiratory (upper airway obstruction, hypoventilation, pulmonary embolism, asthma, COPD exacerbation, pulmonary edema). Circulatory (tension pneumothorax, pericardial tamponade, PE, hemorrhage, sepsis). Electrolyte abnormalities (hypokalemia or hyperkalemia, hypomagnesemia or hypermagnesemia, hypocalcemia). Medications (tricyclic antidepressants, digoxin, theophylline, calcium channel blockers). Drug abuse (cocaine, heroin, amphetamines). Toxins (carbon monoxide, cyanide). Environmental (drowning/near-drowning, electrocution, lightning, hypothermia or hyperthermia, venomous snakes).

CARDIAC ENLARGEMENT7 ICD-9CM # 429.3 CARDIOMEGALY, IDIOPATHIC 746.89 CARDIOMEGALY, CONGENITAL 402.0 CARDIOMEGALY, MALIGNANT 402.1 CARDIOMEGALY, BENIGN

CARDIAC CHAMBER ENLARGEMENT Chronic volume overload Mitral or aortic regurgitation. Left-to-right shunt (PDA, VSD, AV fistula). Cardiomyopathy Ischemic. Nonischemic. Decompensated pressure overload Aortic stenosis. Hypertension.

Pericardial effusion with or without tamponade. Effusive constrictive disease. Pericardial cyst, loculated effusion.

PSEUDOCARDIOMEGALY Epicardial fat. Chest wall deformity (pectus excavatum, straight back syndrome). Low lung volumes. AP chest x-ray. Mediastinal tumor, cyst.

CARDIAC MURMURS ICD-9CM # CODE VARIES WITH SPECIFIC DISORDER

SYSTOLIC Mitral regurgitation (MR). Tricuspid regurgitation (TR). Ventricular septal defect (VSD). Aortic stenosis (AS). Idiopathic hypertrophic subaortic stenosis (IHSS). Pulmonic stenosis (PS). Innocent murmur of childhood. Coarctation of aorta. Mitral valve prolapse (MVP).

DIASTOLIC Aortic regurgitation (AR). Atrial myxoma. Mitral stenosis (MS). Pulmonary artery branch stenosis. Tricuspid stenosis (TS).

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Cavitary Lesion on Chest X-Ray Graham Steell murmur (diastolic decrescendo murmur heard in severe pulmonary hypertension). Pulmonic regurgitation (PR). Severe mitral regurgitation (MR). Austin Flint murmur (diastolic rumble heard in severe AR). Severe VSD and patent ductus arteriosus.

CONTINUOUS Patent ductus arteriosus. Pulmonary AV fistula.

CAVITARY LESION ON CHEST X-RAY9 ICD-9CM # 793.1 CHEST X-RAY LUNG SHADOW

NECROTIZING INFECTIONS

Chest Pain (Pleuritic) Granulomatous angiitis. Takayasu’s disease. Arteritis associated with amphetamine, cocaine, or phenylpropanolamine. Syphilis, mucormycosis. Sjögren’s syndrome. Behçet’s syndrome.

CARDIAC DISORDERS Rheumatic heart disease. Mural thrombus. Arrhythmias. Mitral valve prolapse. Prosthetic heart valve. Endocarditis. Myxoma. Paradoxical embolus.

HEMATOLOGIC DISORDERS

CHEST PAIN (NONPLEURITIC)4

Bland infarction (with or without superimposed infection). Lung contusion.

ICD-9CM # 786.50 CHEST PAIN NOS 786.59 CHEST DISCOMFORT

S. aureus, anaerobes, others.

VASCULITIS Wegener’s granulomatosis, periarteritis.

NEOPLASMS Bronchogenic carcinoma, metastatic carcinoma, lymphoma.

MISCELLANEOUS LESIONS Cysts, blebs, bullae, or pneumatocele with or without fluid collections. Sequestration. Empyema with air-fluid level. Bronchiectasis.

CEREBROVASCULAR DISEASE, ISCHEMIC23 ICD-9CM # 437.9

VASCULAR DISORDERS Large-vessel atherothrombotic disease. Lacunar disease. Arterial-to-arterial embolization. Carotid or vertebral artery dissection. Fibromuscular dysplasia. Migraine. Venous thrombosis. Radiation. Complications of arteriography. Multiple, progressive intracranial arterial occlusions.

INFLAMMATORY DISORDERS Giant cell arteritis. Polyarteritis nodosa. Systemic lupus erythematosus.

Cardiac: myocardial ischemia/infarction, myocarditis. Esophageal: spasm, esophagitis, ulceration, neoplasm, achalasia, diverticula, foreign body. Referred pain from subdiaphragmatic GI structures. Gastric and duodenal: hiatal hernia, neoplasm, PUD. Gallbladder and biliary: cholecystitis, cholelithiasis, impacted stone, neoplasm. Pancreatic: pancreatitis, neoplasm. Dissecting aortic aneurysm. Pain originating from skin, breasts, and musculoskeletal structures: herpes zoster, mastitis, cervical spondylosis. Mediastinal tumors: lymphoma, thymoma. Pulmonary: neoplasm, pneumonia, pulmonary embolism/ infarction. Psychoneurosis. Chest pain associated with mitral valve prolapse.

CHEST PAIN (PLEURITIC) ICD-9CM # 786.52 CHEST PAIN, PLEURITIC Cardiac: pericarditis, postpericardiotomy/Dressler’s syndrome. Pulmonary: pneumothorax, hemothorax, embolism/infarction, pneumonia, empyema, neoplasm, bronchiectasis, pneumomediastinum, TB, carcinomatous effusion. GI: liver abscess, pancreatitis, esophageal rupture, Whipple’s disease with associated pericarditis or pleuritis. Subdiaphragmatic abscess. Pain originating from skin and musculoskeletal tissues: costochondritis, chest wall trauma, fractured rib, interstitial fibrositis, myositis, strain of pectoralis muscle, herpes zoster, soft tissue and bone tumors. Collagen vascular diseases with pleuritis. Psychoneurosis. Familial Mediterranean fever.

DIFFERENTIAL DIAGNOSIS

Thrombotic thrombocytopenic purpura. Sickle cell disease. Hypercoagulable states. Polycythemia. Thrombocytosis. Leukocytosis. Lupus anticoagulant.

CAVITARY INFARCTION

SEPTIC EMBOLISM

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Section II

Bacteria: anaerobes, Staphylococcus aureus, enteric gram-negative bacteria, Pseudomonas aeruginosa, Legionella species, Haemophilus influenzae, Streptococcus pyogenes, Streptococcus pneumoniae (?), Rhodococcus, Actinomyces. Mycobacteria: Mycobacterium tuberculosis, Mycobacterium kansasii, MAI. Bacteria-like: Nocardia species. Fungi: Coccidioides immitis, Histoplasma capsulatum, Blastomyces hominis, Aspergillus species, Mucor species. Parasitic: Entamoeba histolytica, Echinococcus, Paragonimus westermani.

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Cholestasis CHOLESTASIS7 ICD-9CM # 574.71

EXTRAHEPATIC Choledocholithiasis. Bile duct stricture. Cholangiocarcinoma. Pancreatic carcinoma. Chronic pancreatitis. Papillary stenosis. Ampullary cancer. Primary sclerosing cholangitis. Choledochal cysts. Parasites (e.g., ascaris, clonorchis). AIDS. Cholangiography. Biliary atresia. Portal lymphadenopathy. Mirizzi’s syndrome.

INTRAHEPATIC Viral hepatitis. Alcoholic hepatitis. Drug induced. Ductopenia syndromes. Primary biliary cirrhosis. Benign recurrent intrahepatic cholestasis. Byler’s disease. Primary sclerosing cholangitis. Alagille’s syndrome. Sarcoid. Lymphoma. Postoperative. Total parenteral nutrition. Alpha-1-antitrypsin deficiency.

CLUBBING ICD-9CM # 781.5 CLUBBING FINGER Pulmonary neoplasm (lung, pleura). Other neoplasm (GI, liver, Hodgkin’s, thymus, osteogenic sarcoma). Pulmonary infectious process (empyema, abscess, bronchiectasis, TB, chronic pneumonitis). Extrapulmonary infectious process (subacute bacterial endocarditis, intestinal TB, bacterial or amebic dysentery, arterial graft sepsis). Pneumoconiosis. Sarcoidosis. Cyanotic congenital heart disease. Endocrine (Graves’ disease, hyperparathyroidism). Inflammatory bowel disease. Celiac disease. Chronic liver disease, cirrhosis. Pulmonary AV malformations. Idiopathic. Thyroid acropachy. Hereditary (pachydermoperiostosis). Chronic trauma (jackhammer operators, machine workers).

CONSTIPATION ICD-9CM # 564.0 Intestinal obstruction. Fecal impaction. Diverticular disease. GI neoplasm.

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Cyanosis Strangulated femoral hernia. Gallstone ileus. Tuberculous stricture. Adhesions. Ameboma. Volvulus. Intussusception. Inflammatory bowel disease. Hematoma of bowel wall, secondary to trauma or anticoagulants. Poor dietary habits: insufficient bulk in diet, inadequate fluid intake. Change from daily routine: travel, hospital admission, physical inactivity. Acute abdominal conditions: renal colic, biliary colic, appendicitis, ischemia. Hypercalcemia or hypokalemia, uremia. Irritable bowel syndrome, anorexia nervosa, depression. Painful anal conditions: hemorrhoids, fissure, stricture. Decreased intestinal peristalsis: old age, spinal cord injuries, myxedema, diabetes, multiple sclerosis, parkinsonism and other neurologic diseases. Drugs: codeine, morphine, antacids with aluminum, verapamil, anticonvulsants, anticholinergics, disopyramide, cholestyramine, alosetron, iron supplements. Hirschsprung’s disease.

COUGH ICD-9CM # 786.2 Infectious process (viral, bacterial). Postinfectious. “Smoker’s cough.” Rhinitis (allergic, vasomotor, postinfectious). Asthma. Exposure to irritants (noxious fumes, smoke, cold air). Drug-induced (especially ACE inhibitors, -blockers). GERD. Interstitial lung disease. Lung neoplasms. Lymphomas, mediastinal neoplasms. Bronchiectasis. Cardiac (CHF, pulmonary edema, mitral stenosis, pericardial inflammation). Recurrent aspiration. Inflammation of larynx, pleura, diaphragm, mediastinum. Cystic fibrosis. Anxiety. Other: pulmonary embolism, foreign body inhalation, aortic aneurysm, Zenker’s diverticulum, osteophytes, substernal thyroid, thyroiditis, PMR.

CYANOSIS ICD-9CM # 782.5 CYANOSIS NOS Congenital heart disease with right-to-left shunt. Pulmonary embolism. Hypoxia. Pulmonary edema. Pulmonary disease (oxygen diffusion and alveolar ventilation abnormalities). Hemoglobinopathies. Decreased cardiac output. Vasospasm. Arterial obstruction. Pulmonary AV fistulas. Elevated hemidiaphragm.

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Delirium

DELIRIUM14 ICD-9CM # 780.09 DELIRIUM NOS 293.0 ACUTE DELIRIUM Anxiolytics (benzodiazepines). Antidepressants (e.g., amitriptyline, doxepin, imipramine). Cardiovascular agents (e.g., methyldopa, digitalis, reserpine, propranolol, procainamide, captopril, disopyramide). Antihistamine. Cimetidine. Corticosteroids. Antineoplastics. Drugs of abuse (alcohol, cannabis, amphetamines, cocaine, hallucinogens, opioids, sedative-hypnotics, phencyclidine).

METABOLIC DISORDERS Hypercalcemia. Hypercarbia. Hypoglycemia. Hyponatremia. Hypoxia.

INFLAMMATORY DISORDERS Sarcoidosis. SLE. Giant cell arteritis.

ORGAN FAILURE Hepatic encephalopathy. Uremia.

NEUROLOGIC DISORDERS Alzheimer’s disease. CVA. Encephalitis (including HIV). Encephalopathies. Epilepsy. Huntington’s disease. Multiple sclerosis. Neoplasms. Normal pressure hydrocephalus. Parkinson’s disease. Pick’s disease. Wilson’s disease.

ENDOCRINE DISORDERS Addison’s disease. Cushing’s disease. Panhypopituitarism.

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Parathyroid disease. Recurrent menstrual psychosis. Sydenham’s chorea. Thyroid disease.

DEFICIENCY STATES Niacin. Thiamine, vitamin B12, and folate.

DIPLOPIA, BINOCULAR ICD-9CM # 368.2 Cranial nerve palsy (3rd, 4th, 6th). Thyroid eye disease. Myasthenia gravis. Decompensated strabismus. Orbital trauma with blow-out fracture. Orbital pseudotumor. Cavernous sinus thrombosis.

DYSPAREUNIA5 ICD-9CM # 625.0 DYSPAREUNIA 608.89 DYSPAREUNIA, MALE 302.76 DYSPAREUNIA, PSYCHOGENIC

INTROITAL Vaginismus. Intact or rigid hymen. Clitoral problems. Vulvovaginitis. Vaginal atrophy: hypoestrogen. Vulvar dystrophy. Bartholin or Skene gland infection. Inadequate lubrication. Operative scarring.

MIDVAGINAL Urethritis. Trigonitis. Cystitis. Short vagina. Operative scarring. Inadequate lubrication.

DEEP Pelvic infection. Uterine retroversion. Ovarian pathology. Gastrointestinal. Orthopedic. Abnormal penile size or shape.

DYSPHAGIA ICD-9CM # 787.2 Esophageal obstruction: neoplasm, foreign body, achalasia, stricture, spasm, esophageal web, diverticulum, Schatzki’s ring. Peptic esophagitis with stricture, Barrett’s stricture. External esophageal compression: neoplasms (thyroid neoplasm, lymphoma, mediastinal tumors), thyroid enlargement, aortic aneurysm, vertebral spurs, aberrant right subclavian artery (dysphagia lusoria). Hiatal hernia, GERD. Oropharyngeal lesions: pharyngitis, glossitis, stomatitis, neoplasms. Hysteria: globus hystericus.

DIFFERENTIAL DIAGNOSIS

PHARMACOLOGIC AGENTS

Dysphagia

Section II

Neoplasm (bronchogenic carcinoma, mediastinal neoplasm, intrahepatic lesion). Substernal thyroid. Infectious process (pneumonia, empyema, TB, subphrenic abscess, hepatic abscess). Atelectasis. Idiopathic. Eventration. Phrenic nerve dysfunction (myelitis, myotonia, herpes zoster). Trauma to phrenic nerve or diaphragm (e.g., surgery). Aortic aneurysm. Intraabdominal mass. Pulmonary infarction. Pleurisy. Radiation therapy. Rib fracture. Superior vena cava syndrome.

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Dyspnea Neurologic and/or neuromuscular disturbances: bulbar paralysis, myasthenia gravis, ALS, multiple sclerosis, parkinsonism, CVA, diabetic neuropathy. Toxins: poisoning, botulism, tetanus, postdiphtheritic dysphagia. Systemic diseases: scleroderma, amyloidosis, dermatomyositis. Candida and herpes esophagitis. Presbyesophagus.

DYSPNEA ICD-9CM # 786.00 Upper airway obstruction: trauma, neoplasm, epiglottitis, laryngeal edema, tongue retraction, laryngospasm, abductor paralysis of vocal cords, aspiration of foreign body. Lower airway obstruction: neoplasm, COPD, asthma, aspiration of foreign body. Pulmonary infection: pneumonia, abscess, empyema, TB, bronchiectasis. Pulmonary hypertension. Pulmonary embolism/infarction. Parenchymal lung disease. Pulmonary vascular congestion. Cardiac disease: ASHD, valvular lesions, cardiac dysrhythmias, cardiomyopathy, pericardial effusion, cardiac shunts. Space-occupying lesions: neoplasm, large hiatal hernia, pleural effusions. Disease of chest wall: severe kyphoscoliosis, fractured ribs, sternal compression, morbid obesity. Neurologic dysfunction: Guillain-Barré syndrome, botulism, polio, spinal cord injury. Interstitial pulmonary disease: sarcoidosis, collagen vascular diseases, DIP, Hamman-Rich pneumonitis, etc. Pneumoconioses: silicosis, berylliosis, etc. Mesothelioma. Pneumothorax, hemothorax, pleural effusion. Inhalation of toxins. Cholinergic drug intoxication. Carcinoid syndrome. Hematologic: anemia, polycythemia, hemoglobinopathies. Thyrotoxicosis, myxedema. Diaphragmatic compression caused by abdominal distention, subphrenic abscess, ascites. Lung resection. Metabolic abnormalities: uremia, hepatic coma, DKA. Sepsis. Atelectasis. Psychoneurosis. Diaphragmatic paralysis.

DYSURIA ICD-9CM # 788.1 DYSURIA 306.53 DYSURIA, PSYCHOGENIC Urinary tract infection. Estrogen deficiency (in postmenopausal female). Vaginitis. Genital infection (e.g., herpes, condyloma). Interstitial cystitis. Chemical irritation (e.g., deodorant aerosols, douches). Meatal stenosis or stricture. Reiter’s syndrome. Bladder neoplasm. GI etiology (diverticulitis, Crohn’s disease). Impaired bladder or sphincter action. Urethral carbuncle.

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Epilepsy Chronic fibrosis posttrauma. Radiation therapy. Prostatitis. Urethritis (gonococcal, Chlamydiae). Behçet’s syndrome. Stevens-Johnson syndrome.

EDEMA, GENERALIZED ICD-9CM # 782.3 EDEMA NOS Congestive heart failure (CHF). Cirrhosis. Nephrotic syndrome. Idiopathic. Acute nephritic syndrome. Myxedema. Medications (NSAIDs, estrogens, vasodilators).

EDEMA, LEG, UNILATERAL14 ICD-9CM # 782.3

WITH PAIN DVT. Postphlebitic syndrome. Popliteal cyst rupture. Gastrocnemius rupture. Cellulitis. Psoas or other abscess.

WITHOUT PAIN DVT. Postphlebitic syndrome. Other venous insufficiency (after saphenous vein harvest, varicosities). Lymphatic obstruction/lymphedema (carcinoma, lymphoma, sarcoidosis, filariasis, retroperitoneal fibrosis).

EDEMA OF LOWER EXTREMITIES ICD-9CM # 782.3 CHF (right-sided). Hepatic cirrhosis. Nephrosis. Myxedema. Lymphedema. Abdominal mass: neoplasm, cyst. Venous compression from abdominal aneurysm. Varicose veins. Bilateral cellulitis. Bilateral thrombophlebitis. Vena cava thrombosis, venous thrombosis. Retroperitoneal fibrosis.

EPILEPSY ICD-9CM # 345.9 EPILEPSY NOS Psychogenic spells. Transient ischemic attack. Hypoglycemia. Syncope. Narcolepsy. Migraine. Paroxysmal vertigo. Arrhythmias. Drug reaction.

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Epistaxis EPISTAXIS ICD-9CM # 784.7

Failure to Thrive Tic douloureux. Cluster headache, “lower-half headache.” Geniculate neuralgia. Anxiety, somatization syndrome. Glossopharyngeal neuralgia. Carotidynia.

FACIAL PARALYSIS16 ICD-9CM # 351.0 FACIAL (7TH NERVE) PALSY

INFECTION Bacterial: otitis media, mastoiditis, meningitis, Lyme disease. Viral: herpes zoster, mononucleosis, varicella, rubella, mumps, Bell’s palsy. Mycobacterial: TB, meningitis, leprosy. Miscellaneous: syphilis, malaria.

TRAUMA

ERECTILE DYSFUNCTION, ORGANIC18 ICD-9CM # 607.84

Temporal bone fracture, facial laceration. Surgery.

NEOPLASM Malignant: squamous cell carcinoma, basal cell and adenocystic tumors, leukemia, parotid neoplasms, metastic tumors. Benign: facial nerve neuroma, vestibular schwannoma, cholesteatoma.

IMMUNOLOGIC Guillain-Barré syndrome, periarteritis nodosa. Reaction to tetanus antiserum.

METABOLIC Hypothyroidism. DM.

EYE PAIN

FAILURE TO THRIVE

ICD-9CM # 379.91

ICD-9CM # 783.4

Foreign body. Herpes zoster. Trauma. Conjunctivitis. Iritis. Iridocyclitis. Uveitis. Blepharitis. Ingrown lashes. Orbital or periorbital cellulitis/abscess. Sinusitis. Headache. Glaucoma. Inflammation of lacrimal gland. Tic douloureux. Cerebral aneurysm. Cerebral neoplasm. Entropion. Retrobulbar neuritis. UV light. Dry eyes. Irritation or inflammation from eyedrops, dust, cosmetics, etc.

FACIAL PAIN ICD-9CM # 784.0 Infection, abscess. Postherpetic neuralgia. Trauma, posttraumatic neuralgia.

MALABSORPTION Pancreatic insufficiency (chronic pancreatitis). Short bowel syndrome. Celiac disease. Other intestinal mucosal abnormalities.

INSUFFICIENT CALORIC INTAKE Food shortage (poverty). Depression. Dementia. Functional (inability to shop or prepare food).

GASTROINTESTINAL IMPEDIMENTS TO TRANSIT Oropharyngeal dysphagia (SP stroke). Esophageal dysphagia (cancer, stricture). Gastric cancer. Pyloric stenosis. Bowel obstruction.

INCREASED NEEDS Addison’s Disease. Hyperthyroidism. CHF. Malignancy. Renal or hepatic disease. COPD. Diabetes mellitus.

DIFFERENTIAL DIAGNOSIS

Neurogenic abnormalities: somatic nerve neuropathy, central nervous system abnormalities. Psychogenic causes: depression, performance anxiety, marital conflict. Endocrine causes: hyperprolactinemia, hypogonadotropic hypogonadism, testicular failure, estrogen excess. Trauma: pelvic fracture, prostate surgery, penile fracture. Systemic disease: diabetes mellitus, renal failure, hepatic cirrhosis. Medications: diuretics, antidepressants, H2 blockers, exogenous hormones, alcohol, antihypertensives, nicotine abuse, finasteride, etc. Structural abnormalities: Peyronie’s disease.

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Section II

Trauma. Medications (nasal sprays, NSAIDs, anticoagulants, antiplatelets). Nasal polyps. Cocaine use. Coagulopathy (hemophilia, liver disease, DIC, thrombocytopenia). Systemic disorders (hypertension, uremia). Infections. Anatomic malformations. Rhinitis. Nasal polyps. Local neoplasms (benign and malignant). Desiccation. Foreign body.

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Fatigue CHRONIC INFECTIONS Tuberculosis. HIV.

FATIGUE ICD-9CM # 780.7 FATIGUE NOS 300.5 FATIGUE PSYCHOGENIC 780.7 CHRONIC FATIGUE SYNDROME Depression. Anxiety, emotional stress. Inadequate sleep. Prolonged physical activity. Anemia. Hypothyroidism. Medications (-blockers, anxiolytics, antidepressants, sedating antihistamines, clonidine, methyldopa). Viral or bacterial infections. Sleep apnea syndrome. Dieting. Renal failure, CHF, COPD, liver disease.

FATTY LIVER ICD-9CM # 571.8 Obesity. Alcohol abuse. Diabetes mellitus. Medications (tetracycline, valproic acid, glucocorticoids, amiodarone, estrogen, methotrexate). Reye’s syndrome. Wilson’s disease. Nonalcoholic steatosis and steatohepatitis.

FLATULENCE AND BLOATING19 ICD-9CM # 787.3 Ingestion of nonabsorbable carbohydrates. Ingestion of carbonated beverages. Malabsorption: pancreatic insufficiency, biliary disease, celiac disease, bacterial overgrowth in small intestine. Lactase deficiency. Irritable bowel syndrome. Anxiety disorders. Food poisoning, giardiasis.

GAIT ABNORMALITY ICD-9CM # 781.2 GAIT ABNORMALITY Parkinsonism. Degenerative joint disease (hips, back, knees). Multiple sclerosis. Trauma, foot pain. CVA. Cerebellar lesions. Infections (tabes, encephalitis, meningitis). Sensory ataxia. Dystonia, cerebral palsy, neuromuscular disorders. Metabolic abnormalities.

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Headache GOITER ICD-9CM # 240.9 241.9 246.1 240.9 241.1 240.0 242.1

GOITER, UNSPECIFIED GOITER, ADENOMATOUS GOITER, CONGENITAL GOITER, NONTOXIC DIFFUSE GOITER, NONTOXIC MULTINODULAR SIMPLE GOITER THYROTOXIC GOITER

Thyroiditis. Toxic multinodular goiter. Graves’ disease. Medications (PTU, methimazole, sulfonamides, sulfonylureas, ethionamide, amiodarone, lithium, etc.). Iodine deficiency. Sarcoidosis, amyloidosis. Defective thyroid hormone synthesis. Resistance to thyroid hormone.

GYNECOMASTIA ICD-9CM # 611.1 GYNECOMASTIA, NONPUERPERAL Physiologic (aging). Drugs (estrogen and estrogen precursors, digitalis, testosterone and exogenous androgens, cimetidine, spironolactone, ketoconazole, amiodarone, ACE inhibitors, isoniazid, phenytoin, methyldopa, metoclopramide, phenothiazine). Increased prolactin level (prolactinoma). Liver disease. Adrenal disease. Thyrotoxicosis. Increased estrogen production (hCG-producing tumor, testicular tumor, bronchogenic carcinoma). Secondary hypogonadism. Primary gonadal failure (trauma, castration, viral orchitis, granulomatous disease). Defects in androgen synthesis. Testosterone deficiency. Klinefelter’s syndrome.

HALITOSIS ICD-9CM # 784.9 Tobacco use. Alcohol use. Dry mouth (mouth breathing, inadequate fluid intake). Foods (onion, garlic, meats, nuts). Disease of mouth or nose (infections, cancer, inflammation). Medications (antihistamines, antidepressants). Systemic disorders (diabetes, uremia). GI disorders (esophageal diverticula, hiatal hernia, GERD, achalasia). Sinusitis. Pulmonary disorders (bronchiectasis, pneumonia, neoplasms, TB).

HEADACHE6 ICD-9CM # 784.0 HEADACHE NOS 307.81 HEADACHE, TENSION 346.2 HEADACHE, CLUSTER 346.9 HEADACHE, MIGRAINE 784.0 HEADACHE, VASCULAR Vascular: migraine, cluster headaches, temporal arteritis, hypertension, cavernous sinus thrombosis. Musculoskeletal: neck and shoulder muscle contraction, strain of extraocular and/or intraocular muscles, cervical spondylosis, temporomandibular arthritis.

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Hearing Loss, Acute

HEARING LOSS, ACUTE14 Infectious: mumps, measles, influenza, herpes simplex, herpes zoster, CMV, mononucleosis, syphilis. Vascular: macroglobulinemia, sickle cell disease, Berger’s disease, leukemia, polycythemia, fat emboli, hypercoagulable states. Metabolic: diabetes, hyperlipoproteinemia. Conductive: cerumen impaction, foreign bodies, otitis media, otitis externa, barotrauma, trauma. Medications: aminoglycosides, loop diuretics, antineoplastics, salicylates, vancomycin. Neoplasm: acoustic neuroma, metastatic neoplasm.

HEARTBURN AND INDIGESTION19 ICD-9CM # 787.1 HEARTBURN 536.8 INDIGESTION Reflux esophagitis. Gastritis. Nonulcer dyspepsia. Functional GI disorder (anxiety disorder, social/environmental stresses). Excessive intestinal gas (ingestion of flatulogenic foods, GI stasis, constipation). Gas entrapment (hepatitis or splenic flexure syndrome). Neoplasm (adenocarcinoma of stomach or esophagus, lymphoma). Gallbladder disease.

HEEL PAIN, PLANTAR13 ICD-9CM # 729.5

SKIN Keratoses. Verruca. Ulcer. Fissure.

CONNECTIVE TISSUE Fat Atrophy. Panniculitis. Dense connective tissue Inflammatory fasciitis. Fibromatosis.

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Enthesopathy. Bursitis. Bone (calcaneus) Stress fracture. Paget’s disease. Benign bone cyst/tumor. Malignant bone tumor. Metabolic bone disease (osteopenia). Nerve Tarsal tunnel. Plantar nerve entrapment. S1 nerve root radiculopathy. Painful peripheral neuropathy.

INFECTION Dermatomycoses. Acute osteomyelitis. Plantar abscess.

MISCELLANEOUS Foreign body. Nonunion calcaneus fracture. Psychogenic. Idiopathic.

HEMATURIA ICD-9CM # 599.7 HEMATURIA, BENIGN (ESSENTIAL) Use the mnemonic TICS: T (trauma): blow to kidney, insertion of Foley catheter or foreign body in urethra, prolonged and severe exercise, very rapid emptying of overdistended bladder. (tumor): hypernephroma, papillary carcinoma of the bladder, prostatic and urethral neoplasms. (toxins): turpentine, phenols, sulfonamides and other antibiotics, cyclophosphamide, NSAIDs. I (infections): glomerulonephritis, TB, cystitis, prostatitis, urethritis, Schistosoma haematobium, yellow fever, blackwater fever. (inflammatory processes): Goodpasture’s syndrome, periarteritis, postirradiation. C (calculi): renal, ureteral, bladder, urethra. (cysts): simple cysts, polycystic disease. (congenital anomalies): hemangiomas, aneurysms, AVM. S (surgery): invasive procedures, prostatic resection, cystoscopy. (sickle cell disease and other hematologic disturbances): hemophilia, thrombocytopenia, anticoagulants. (somewhere else): bleeding genitals, factitious (drug addicts).

HEMATURIA, CAUSED BY AGE AND SEX ICD-9CM # 599.7 HEMATURIA BENIGN (ESSENTIAL) OTHER CODES FOR HEMATURIA VARY WITH CAUSE OF HEMATURIA

60 YR AND OLDER (WOMEN) Acute urinary tract infection. Bladder cancer. Vaginal trauma or irritation. Urolithiasis.

60 YR AND OLDER (MEN) Acute urinary tract infection. Benign prostatic hyperplasia. Bladder cancer. Urolithiasis. Trauma.

DIFFERENTIAL DIAGNOSIS

ICD-9CM # 388.2

Hematuria, Caused by Age and Sex

Section II

Infections: meningitis, encephalitis, brain abscess, sepsis, sinusitis, osteomyelitis, parotitis, mastoiditis. Cerebral neoplasm. Subdural hematoma. Cerebral hemorrhage/infarct. Normal pressure hydrocephalus (NPH). Postlumbar puncture. Cerebral aneurysm, arteriovenous malformations. Posttrauma. Dental problems: abscess, periodontitis, poorly fitting dentures. Trigeminal neuralgia, glossopharyngeal neuralgia. Otitis and other ear diseases. Glaucoma and other eye diseases. Metabolic: uremia, carbon monoxide inhalation, hypoxia. Pheochromocytoma, hypoglycemia, hypothyroidism. Effort induced: benign exertional headache, cough, headache, coital cephalalgia. Drugs: alcohol, nitrates, histamine antagonists. Paget’s disease of the skull. Emotional, psychiatric.

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Hemiparesis/Hemiplegia

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Hypercalcemia

HEMIPARESIS/HEMIPLEGIA

HICCUPS11

ICD-9CM # 436.0 ACQUIRED DUE TO ACUTE CVA, FLACCID 436.1 ACQUIRED DUE TO CVA, ACUTE, SPASTIC

ICD-9CM # 786.8

CVA. Transient ischemic attack. Cerebral neoplasm. Multiple sclerosis or other demyelinating disorder. CNS infection. Migraine. Hypoglycemia. Subdural hematoma. Vasculitis. Todd’s paralysis. Epidural hematoma. Metabolic (hyperosmolar state, electrolyte imbalance). Psychiatric disorders. Congenital disorders. Leukodystrophies.

HEMOPTYSIS ICD-9CM # 786.3

CARDIOVASCULAR Pulmonary embolism/infarction. Left ventricular failure. Mitral stenosis. AV fistula. Severe hypertension. Erosion of aortic aneurysm.

PULMONARY Neoplasm (primary or metastatic). Infection. Pneumonia: Streptococcus pneumoniae, Klebsiella pneumoniae, Staphylococcus aureus, Legionella pneumophila. Bronchiectasis. Abscess. TB. Bronchitis. Fungal infections (aspergillosis, coccidioidomycosis). Parasitic infections (amebiasis, ascariasis, paragonimiasis). Vasculitis: Wegener’s granulomatosis, Churg-Strauss syndrome, Henoch-Schönlein purpura. Goodpasture’s syndrome. Trauma (needle biopsy, foreign body, right-sided heart catheterization, prolonged and severe cough). Bullous emphysema. Pulmonary sequestration. Pulmonary AV fistula. SLE. Idiopathic pulmonary hemosiderosis. Drugs: aspirin, anticoagulants, penicillamine. Pulmonary hypertension. Mediastinal fibrosis.

OTHER Epistaxis, trauma. Laryngeal bleeding (laryngitis, laryngeal neoplasm). Hematologic disorders (clotting abnormalities, DIC, thrombocytopenia).

TRANSIENT HICCUPS Sudden excitement, emotion. Gastric distention. Esophageal obstruction. Alcohol ingestion. Sudden change in temperature.

PERSISTENT OR CHRONIC HICCUPS Toxic/metabolic: uremia, DM, hyperventilation, hypocalcemia, hypokalemia, hyponatremia, gout, fever. Drugs: benzodiazepines, steroids, -methyldopa, barbiturates. Surgery/general anesthesia. Thoracic/diaphragmatic disorders: pneumonia, lung cancer, asthma, pleuritis, pericarditis, myocardial infarction, aortic aneurysm, esophagitis, esophageal obstruction, diaphragmatic hernia or irritation. Abdominal disorders: gastric ulcer or cancer, hepatobiliary or pancreatic disease, IBD, bowel obstruction, intraabdominal or subphrenic abscess, prostatic infection or cancer. Central nervous system disorders: traumatic, infectious, vascular, structural. Ear, nose, and throat disorders: pharyngitis, laryngitis, tumor, irritation of auditory canal. Psychogenic disorders. Idiopathic disorders.

HOARSENESS ICD-9CM # 784.49 Allergic rhinitis. Infections (laryngitis, epiglottitis, tracheitis, croup). Vocal cord polyps. Voice strain. Irritants (tobacco smoke). Vocal cord trauma (intubation, surgery). Neoplastic involvement of vocal cord (primary or metastatic). Neurologic abnormalities (multiple sclerosis, ALS, parkinsonism). Endocrine abnormalities (puberty, menopause, hypothyroidism). Other (laryngeal webs or cysts, psychogenic, muscle tension abnormalities).

HYPERCALCEMIA ICD-9CM # 275.42 HYPERCALCEMIA DISORDER Malignancy: increased bone resorption via osteoclast-activating factors, secretion of PTH-like substances, prostaglandin E2, direct erosion by tumor cells, transforming growth factors, colony-stimulating activity. Hypercalcemia is common in the following neoplasms: Solid tumors: breast, lung, pancreas, kidneys, ovary. Hematologic cancers: myeloma, lymphosarcoma, adult. T-cell lymphoma, Burkitt’s lymphoma. Hyperparathyroidism: increased bone resorption, GI absorption, and renal absorption; etiology: Parathyroid hyperplasia, adenoma. Hyperparathyroidism or renal failure with secondary hyperparathyroidism. Granulomatous disorders: increased GI absorption (e.g., sarcoidosis).

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Hyperhidrosis Paget’s disease: increased bone resorption, seen only during periods of immobilization. Vitamin D intoxication, milk-alkali syndrome; increased GI absorption. Thiazides: increased renal absorption. Other causes: familial hypocalciuric hypercalcemia, thyrotoxicosis, adrenal insufficiency, prolonged immobilization, vitamin A intoxication, recovery from acute renal failure, lithium administration, pheochromocytoma, disseminated SLE.

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Hypocalcemia Spinal Cord transection. Syringomyelia. Changes in blood flow Mallucci syndrome. Arteriovenous fistula. Klippel-Trenaunay syndrome. Glomus tumor. Blue rubber bleb nevus syndrome.

HYPERHIDROSIS3

HYPERKALEMIA

ICD-9CM # 780.8 HYPERHIDROSIS

ICD-9CM # 276.7

CORTICAL

Drugs Antipyretics. Emetics. Insulin. Meperidine. Exercise Infection Defervescence. Chronic illness. Metabolic Debility. Diabetes mellitus. Hyperpituitarism. Hyperthyroidism. Hypoglycemia. Obesity. Porphyria. Cardiovascular Heart failure. Shock. Vasomotor Cold injury. Raynaud’s phenomenon. Rheumatoid arthritis. Neurologic Abscess. Familial dysautonomia. Postencephalitic. Tumor. Miscellaneous Chédiak-Higashi syndrome. Compensatory. Phenylketonuria. Pheochromocytoma. Vitiligo. Medullary Physiologic gustatory sweating. Encephalitis. Granulosis rubra nasi. Syringomyelia. Thoracic sympathetic trunk injury.

HYPOCALCEMIA ICD-9CM # 275.41 Renal insufficiency: hypocalcemia caused by: Increased calcium deposits in bone and soft tissue secondary to increased serum PO423 level. Decreased production of 1,25-dihydroxyvitamin D. Excessive loss of 25-OHD (nephrotic syndrome). Hypoalbuminemia: each decrease in serum albumin (g/L) will decrease serum calcium by 0.8 mg/dl but will not change free (ionized) calcium. Vitamin D deficiency: Malabsorption (most common cause). Inadequate intake. Low sun exposure.

DIFFERENTIAL DIAGNOSIS

HYPOTHALAMIC

Pseudohyperkalemia. Hemolyzed specimen. Severe thrombocytosis (platelet count 1 million/ml). Severe leukocytosis (white blood cell count 100,000/ml). Fist clenching during phlebotomy. Excessive potassium intake (often in setting of impaired excretion). Potassium replacement therapy. High-potassium diet. Salt substitutes with potassium. Potassium salts of antibiotics. Decreased renal excretion. Potassium-sparing diuretics (e.g., spironolactone, triamterene, amiloride). Renal insufficiency. Mineralocorticoid deficiency. Hyporeninemic hypoaldosteronism (DM). Tubular unresponsiveness to aldosterone (e.g., SLE, multiple myeloma, sickle cell disease). Type 4 RTA. ACE inhibitors. Heparin administration. NSAIDs. Trimethoprim-sulfamethoxazole. -Blockers. Pentamidine. Redistribution (excessive cellular release). Acidemia (each 0.1 decrease in pH increases the serum potassium by 0.4 to 0.6 mEq/L). Lactic acidosis and ketoacidosis cause minimal redistribution. Insulin deficiency. Drugs (e.g., succinylcholine, markedly increased digitalis level, arginine, -adrenergic blockers). Hypertonicity. Hemolysis. Tissue necrosis, rhabdomyolysis, burns. Hyperkalemic periodic paralysis.

Section II

Emotional. Familial dysautonomia. Congenital ichthyosiform erythroderma. Epidermolysis bullosa. Nail-patella syndrome. Jadassohn-Lewandowsky syndrome. Pachyonychia congenita. Palmoplantar keratoderma.

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Hypocapnia Decreased production of 1,25-dihydroxyvitamin D (vitamin D-dependent rickets, renal failure). Decreased production of 25-OHD (parenchymal liver disease). Accelerated 25-OHD catabolism (phenytoin, phenobarbital). End-organ resistance to 1,25-dihydroxyvitamin D. Hypomagnesemia: hypocalcemia caused by: Decreased PTH secretion. Inhibition of PTH effect on bone. Pancreatitis, hyperphosphatemia, osteoblastic metastases: hypocalcemia is secondary to increased calcium deposits (bone, abdomen). Pseudohypoparathyroidism (PHP): autosomal recessive disorder characterized by short stature, shortening of metacarpal bones, obesity, and mental retardation; the hypocalcemia is secondary to congenital end-organ resistance to PTH. Idiopathic hypoparathyroidism, surgical removal of parathyroids (e.g., neck surgery). “Hungry bones syndrome”: rapid transfer of calcium from plasma into bones after removal of a parathyroid tumor. Sepsis. Massive blood transfusion (as a result of EDTA in blood).

HYPOCAPNIA ICD-9CM # 786.01 Hyperventilation. Pneumonia, pneumonitis. Fever, sepsis. Medications (salicylates, -adrenergic agonists, progesterone, methylxanthines). Pulmonary disease (asthma, interstitial fibrosis). Pulmonary embolism. Hepatic failure. Metabolic acidosis. High altitude. CHF. Pain. CNS lesions.

HYPOKALEMIA ICD-9CM # 276.8 Cellular shift (redistribution) and undetermined mechanisms. Alkalosis (each 0.1 increase in pH decreases serum potassium by 0.4 to 0.6 mEq/L). Insulin administration. Vitamin B12 therapy for megaloblastic anemias, acute leukemias. Hypokalemic periodic paralysis: rare familial disorder manifested by recurrent attacks of flaccid paralysis and hypokalemia. -Adrenergic agonists (e.g., terbutaline), decongestants, bronchodilators, theophylline, caffeine. Barium poisoning, toluene intoxication, verapamil intoxication, chloroquine intoxication. Correction of digoxin intoxication with digoxin antibody fragments (Digibind). Increased renal excretion. Drugs: Diuretics, including carbonic anhydrase inhibitors (e.g., acetazolamide). Amphotericin B. High-dose sodium penicillin, nafcillin, ampicillin, or carbenicillin.

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Hypotension, Postural Cisplatin. Aminoglycosides. Corticosteroids, mineralocorticoids. Foscarnet sodium. RTA: distal (type 1) or proximal (type 2). Diabetic ketoacidosis (DKA), ureteroenterostomy. Magnesium deficiency. Postobstruction diuresis, diuretic phase of ATN. Osmotic diuresis (e.g., mannitol). Bartter’s syndrome: hyperplasia of juxtaglomerular cells leading to increased renin and aldosterone, metabolic alkalosis, hypokalemia, muscle weakness, and tetany. Increased mineralocorticoid activity (primary or secondary aldosteronism), Cushing’s syndrome. Chronic metabolic alkalosis from loss of gastric fluid (increased renal potassium secretion). GI loss. Vomiting, nasogastric suction. Diarrhea. Laxative abuse. Villous adenoma. Fistulas. Inadequate dietary intake (e.g., anorexia nervosa). Cutaneous loss (excessive sweating). High dietary sodium intake, excessive use of licorice.

HYPOMAGNESEMIA ICD-9CM # 275.2 GI and nutritional Defective GI absorption (malabsorption). Inadequate dietary intake (e.g., alcoholics). Parenteral therapy without magnesium. Chronic diarrhea, villous adenoma, prolonged nasogastric suction, fistulas (small bowel, biliary). Excessive renal losses Diuretics. RTA. Diuretic phase of ATN. Endocrine disturbances (DKA, hyperaldosteronism, hyperthyroidism, hyperparathyroidism), SIADH, Bartter’s syndrome, hypercalciuria, hypokalemia. Cisplatin, alcohol, cyclosporine, digoxin, pentamidine, mannitol, amphotericin B, foscarnet, methotrexate. Antibiotics (gentamicin, ticarcillin, carbenicillin). Redistribution: hypoalbuminemia, cirrhosis, administration of insulin and glucose, theophylline, epinephrine, acute pancreatitis, cardiopulmonary bypass. Miscellaneous: sweating, burns, prolonged exercise, lactation, “hungry-bones” syndrome.

HYPOTENSION, POSTURAL ICD-9CM # 458.0 Antihypertensive medications (especially -blockers, diuretics, ACE inhibitors). Volume depletion (hemorrhage, dehydration). Impaired cardiac output (constrictive pericarditis, aortic stenosis). Peripheral autonomic dysfunction (DM, Guillain-Barré). Idiopathic orthostatic hypotension. Central autonomic dysfunction (Shy-Drager syndrome). Peripheral venous disease. Adrenal insufficiency.

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Impotence

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Joint Pain, Anterior Hip, Medial Thigh, Knee

IMPOTENCE15

ISCHEMIC COLITIS, NONOCCLUSIVE11

ICD-9CM # 302.72 PSYCHOSEXUAL 607.84 ORGANIC 997.99 ORGANIC POSTPROSTATECTOMY

ICD-9CM # 557.1

INSOMNIA19

Anxiety disorder, psychophysiologic insomnia. Depression. Drugs (e.g., caffeine, amphetamines, cocaine), hypnotic-dependent sleep disorder. Pain, fibromyalgia. Inadequate sleep hygiene. Restless leg syndrome. Obstructive sleep apnea. Sleep bruxism. Medical illness (e.g., GERD, sleep-related asthma, parkinsonism and movement disorders). Narcolepsy. Other: periodic leg movement of sleep, central sleep apnea, REM behavioral disorder.

INTESTINAL PSEUDOOBSTRUCTION22 ICD-9CM # 560.1 ADYNAMIC INTESTINAL OBSTRUCTION 564.9 INTESTINAL DISORDER, FUNCTIONAL

“PRIMARY” (IDIOPATHIC INTESTINAL PSEUDOOBSTRUCTION) Hollow visceral myopathy: Familial. Sporadic. Neuropathic: Abnormal myenteric plexus. Normal myenteric plexus.

SECONDARY Scleroderma. Myxedema. Amyloidosis. Muscular dystrophy. Hypokalemia. Chronic renal failure. Diabetes mellitus. Drug toxicity caused by: Anticholinergics. Opiate narcotics. Ogilvie’s syndrome.

Small vessel obstruction Collagen-vascular disease. Vasculitis, diabetes. Nonocclusive hypoperfusion Hemorrhage. CHF, MI, arrhythmias. Sepsis. Vasoconstricting agents: vasopressin, ergot. Increased viscosity: polycythemia, sickle cell disease, thrombocytosis.

INCREASED DEMAND ON MARGINAL BLOOD FLOW Increased motility Mass lesion, stricture. Constipation. Increased intraluminal pressure Bowel obstruction. Colonoscopy. Barium enema.

JOINT PAIN, ANTERIOR HIP, MEDIAL THIGH, KNEE16 ICD-9CM # 719.4 ADD 5TH DIGIT 0 SITE NOS 1 SHOULDER REGION 2 UPPER ARM (ELBOW, HUMERUS) 3 FOREARM (RADIUS, WRIST, ULNA) 4 HAND 5 PELVIC REGION AND THIGH 6 LOWER LEG (FIBULA, PATELLA, TIBIA) 7 ANKLE AND/OR FOOT

ACUTE Acute rheumatic fever. Adductor muscle strain. Avascular necrosis. Crystal arthritis. Femoral artery (pseudo) aneurysm. Fracture (femoral neck or intertrochanteric). Hemarthrosis. Hernia. Herpes zoster. Iliopectineal bursitis. Iliopsoas tendinitis. Inguinal lymphadenitis. Osteomalacia. Painful transient osteoporosis of hip. Septic arthritis.

SUBACUTE AND CHRONIC Adductory muscle strain. Amyloidosis. Acute rheumatic fever. Femoral artery aneurysm. Hernia (inguinal or femoral). Iliopectineal bursitis. Iliopsoas tendinitis. Inguinal lymphadenopathy. Osteochondromatosis. Osteomyelitis. Osteitis deformans (Paget’s disease). Osteomalacia (pseudofracture).

DIFFERENTIAL DIAGNOSIS

ICD-9CM # 780.52 INSOMNIA NOS 307.42 INSOMNIA, CHRONIC ASSOCIATED WITH ANXIETY OR DEPRESSION 780.51 INSOMNIA WITH SLEEP APNEA

ACUTE DIMINUTION OF COLONIC INTRAMURAL BLOOD FLOW

Section II

Psychogenic. Endocrine: hyperprolactinemia, DM, Cushing’s syndrome, hypothyroidism or hyperthyroidism, abnormality of hypothalamic-pituitary-testicular axis. Vascular: arterial insufficiency, venous leakage, AV malformation, local trauma. Medications. Neurogenic: autonomic or sensory neuropathy, spinal cord trauma or tumor, CVA, multiple sclerosis, temporal lobe epilepsy. Systemic illness: renal failure, COPD, cirrhosis of liver, myotonic dystrophy. Peyronie’s disease. Prostatectomy.

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Joint Pain, Hip, Lateral Thigh Postherpetic neuralgia. Sterile synovitis (e.g., rheumatoid arthritis, psoriatic, systemic lupus erythematosus).

JOINT PAIN, HIP, LATERAL THIGH16 ICD-9CM # 959.6 HIP INJURY 719.95 HIP JOINT DISORDER 843.9 HIP STRAIN

ACUTE Herpes zoster. Iliotibial tendinitis. Impacted fracture of femoral neck. Lateral femoral cutaneous neuropathy (meralgia paresthetica). Radiculopathy: L4-5. Trochanteric avulsion fracture (greater trochanter). Trochanteric bursitis. Trochanteric fracture.

SUBACUTE AND CHRONIC Lateral femoral cutaneous neuropathy (meralgia paresthetica). Osteomyelitis. Postherpetic neuralgia. Radiculopathy: L4-5. Tumors.

JOINT PAIN, POSTERIOR HIPS, THIGH, BUTTOCKS16 ICD-9CM # 719.4 ADD 5TH DIGIT 0 SITE NOS 1 SHOULDER REGION 2 UPPER ARM (ELBOW, HUMERUS) 3 FOREARM (RADIUS, WRIST, ULNA) 4 HAND 5 PELVIC REGION AND THIGH 6 LOWER LEG (FIBULA, PATELLA, TIBIA) 7 ANKLE AND/OR FOOT

ACUTE Gluteal muscle strain. Herpes zoster. Ischial bursitis. Ischial or sacral fracture. Osteomalacia (pseudofracture). Sciatic neuropathy. Radiculopathy: L5-S1.

SUBACUTE AND CHRONIC Gluteal muscle strain. Ischial bursitis. Lumbar spinal stenosis. Osteoarthritis of hip. Osteitis deformans (Paget’s disease). Osteomyelitis. Osteochondromatosis. Osteomalacia (pseudofracture). Postherpetic neuralgia. Radiculopathy: L5-S1. Tumors.

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Knee Pain JOINT SWELLING ICD-9CM # 719.0 ADD 5TH DIGIT 0 SITE NOS 1 SHOULDER REGION 2 UPPER ARM (ELBOW, HUMERUS) 3 FOREARM (RADIUS, WRIST, ULNA) 4 HAND 5 PELVIC REGION AND THIGH 6 LOWER LEG (FIBULA, PATELLA, TIBIA) 7 ANKLE AND/OR FOOT Trauma. Osteoarthritis. Gout. Pyogenic arthritis. Pseudogout. Rheumatoid arthritis. Viral syndrome.

KNEE PAIN16 ICD-9CM # 844.1 COLLATERAL LIGAMENT SPRAIN, MEDIAL 844.2 CRUCIATE LIGAMENT SPRAIN 716.96 KNEE INFLAMMATION 959.7 KNEE INJURY 718.86 KNEE INSTABILITY 836.1 LATERAL MENISCUS TEAR 836.0 MEDIAL MENISCUS TEAR 844.8 PATELLAR SPRAIN 719.56 KNEE STIFFNESS 719.06 KNEE SWELLING

DIFFUSE Articular. Anterior. Prepatellar bursitis. Patellar tendon enthesopathy. Chondromalacia patellae. Patellofemoral osteoarthritis. Cruciate ligament injury. Medial plica syndrome.

MEDIAL Anserine bursitis. Spontaneous osteonecrosis. Osteoarthritis. Medial meniscal tear. Medial collateral ligament bursitis. Referred pain from hip and L3. Fibromyalgia.

LATERAL Iliotibial band syndrome. Meniscal cyst. Lateral meniscal tear. Collateral ligament. Peroneal tenosynovitis.

POSTERIOR Popliteal cyst (Baker’s cyst). Tendinitis. Aneurysms, ganglions, sarcoma.

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Leg Cramps, Nocturnal LEG CRAMPS, NOCTURNAL ICD-9CM # 729.82 MUSCLE CRAMPS

Lymphadenopathy TUMORS Basal cell carcinoma, squamous cell carcinoma, melanoma. Mycosis fungoides, Kaposi’s sarcoma, metastatic neoplasms.

TRAUMA Pressure ulcer. Burns, cold injury, radiation dermatitis. Insect bites. Factitial, excessive pressure.

NEUROPATHIC Diabetic trophic ulcers. Tabes dorsalis, syringomyelia.

DRUGS Warfarin, IV colchicine extravasation, methotrexate, halogens, ergotism, hydroxyurea.

PANNICULITIS Weber-Christian disease. Pancreatic fat necrosis, alpha-antitrypsinase deficiency.

LYMPHADENOPATHY7

ICD-9CM # 729.82 MUSCLE CRAMPS

ICD-9CM # 785.6

LEG ULCERS16 ICD-9CM # 440.23 LOWER LIMB, ARTERIOSCLEROTIC 707.1 LOWER LIMB, CHRONIC 707.1 LOWER LIMB, NEUROGENIC 250.70 LOWER LIMB, CHRONIC DIABETES MELLITUS TYPE II 250.71 LOWER LIMB, CHRONIC, DIABETES MELLITUS TYPE I

VASCULAR Arterial: arteriosclerosis, thromboangiitis obliterans, AV malformation, cholesterol emboli. Venous: superficial varicosities, incompetent perforators, DVT, lymphatic abnormalities.

VASCULITIS HEMATOLOGIC Sickle cell anemia, thalassemia, polycythemia vera, leukemia, cold agglutinin disease. Macroglobulinemia, protein C and protein S deficiency, cryoglobulinemia, lupus anticoagulant, antiphospholipid syndrome.

INFECTIOUS Fungus: blastomycosis, coccidioidomycosis, histoplasmosis, sporotrichosis. Bacterial: furuncle, ecthyma, septic emboli. Protozoal: leishmaniasis.

METABOLIC Necrobiosis lipoidica diabeticorum. Localized bullous pemphigoid. Gout, calcinosis cutis, Gaucher’s disease.

GENERALIZED AIDS. Lymphoma: Hodgkin’s disease, non-Hodgkin’s lymphoma. Leukemias, reticuloendotheliosis. Viral infections (infectious mononucleosis, CMV). Diffuse skin infection: generalized furunculosis, multiple tick bites. Parasitic infections: toxoplasmosis, filariasis, leishmaniasis, Chagas’ disease. Serum sickness. Collagen vascular diseases (RA, SLE). Dengue (arbovirus infection). Sarcoidosis and other granulomatous diseases. Drugs: INH, hydantoin derivatives, antithyroid and antileprosy drugs. Secondary syphilis. Hyperthyroidism, lipid-storage diseases.

LOCALIZED Cervical nodes Infections of the head, neck, ears, sinuses, scalp, pharynx. Lymphoma. TB. Malignancy of head and neck. Scalene/supraclavicular nodes Lymphoma. Lung neoplasm. Bacterial or fungal infection of thorax or retroperitoneum. GI malignancy. Axillary nodes Infections of hands and arms. Cat-scratch disease. Neoplasm (lymphoma, melanoma, breast carcinoma). Brucellosis. Epitrochlear nodes Infections of the hand. Lymphoma. Tularemia. Sarcoidosis, secondary syphilis (usually bilateral). Inguinal nodes Infections of leg or foot, folliculitis (pubic hair). LGV, syphilis.

DIFFERENTIAL DIAGNOSIS

LEG PAIN WITH EXERCISE Shin splints. Arteriosclerosis obliterans. Neurogenic (spinal cord compression or ischemia). Venous claudication. Popliteal cyst. DVT. Thromboangiitis obliterans. Adventitial cysts. Popliteal artery entrapment syndrome. McArdle’s syndrome.

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Section II

Diabetic neuropathy. Medications. Electrolyte abnormalities (hypokalemia, hyponatremia, hypocalcemia, hyperkalemia, hypophosphatemia). Respiratory alkalosis. Uremia. Hemodialysis. Peripheral nerve injury. ALS. Alcohol use. Heat cramps. Vitamin B12 deficiency. Hyperthyroidism. Contractures. DVT. Hypoglycemia. Peripheral vascular insufficiency. Baker cyst.

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Mediastinal Masses or Widening on Chest X-Ray ● Lymphoma. Pelvic malignancy. Pasteurella pestis. Hilar nodes Sarcoidosis. TB. Lung carcinoma. Fungal infections, systemic. Mediastinal nodes Sarcoidosis. Lymphoma. Lung neoplasm. TB. Mononucleosis. Histoplasmosis. Abdominal/retroperitoneal nodes Lymphoma. TB. Neoplasm (ovary, testes, prostate, and other malignancies).

Muscle Weakness METASTATIC NEOPLASMS ICD-9CM # 198.5 198.3 197.7 197.0 To: Bone Breast Lung Prostate Thyroid Kidney Bladder Endometrium Cervix Melanoma

BONE AND BONE MARROW BRAIN AND SPINAL CORD LIVER LUNG

To: Brain Lung Breast Melanoma GU tract Colon Sinuses Sarcoma Skin Thyroid

To: Liver Colon Stomach Pancreas Breast Lymphomas Bronchus Lung Sarcoma Kidney

To: Lung Breast Colon Kidney Testis Stomach Thyroid Melanoma

MONONEUROPATHY ICD-9CM # 355.9

MEDIASTINAL MASSES OR WIDENING ON CHEST X-RAY ICD-9CM # 785.6 ADENOPATHY 519.3 DISEASE NEC 793.2 SHIFT (CXR) Lymphoma: Hodgkin’s disease and non-Hodgkin’s lymphoma. Sarcoidosis. Vascular: aortic aneurysm, ectasia or tortuosity of aorta or bronchocephalic vessels. Carcinoma: lungs, esophagus. Esophageal diverticula. Hiatal hernia. Achalasia. Prominent pulmonary outflow tract: pulmonary hypertension, pulmonary embolism, right-to-left shunts. Trauma: mediastinal hemorrhage. Pneumomediastinum. Lymphadenopathy caused by silicosis and other pneumoconioses. Leukemias. Infections: TB, viral (rare), Mycoplasma (rare), fungal, tularemia. Substernal thyroid. Thymoma. Teratoma. Bronchogenic cyst. Pericardial cyst. Neurofibroma, neurosarcoma, ganglioneuroma.

MESENTERIC ISCHEMIA, NONOCCLUSIVE14 ICD-9CM # 557.0 MESENTERIC ARTERY EMBOLISM OR INFARCTION 557.1 MESENTERIC ARTERY INSUFFICIENCY, CHRONIC 902.39 MESENTERIC VEIN INJURY Cardiovascular disease resulting in low-flow states (CHF, cardiogenic shock, post cardiopulmonary bypass, dysrhythmias). Septic shock. Drug induced (cocaine, vasopressors, ergot alkaloid poisoning).

Herpes zoster. Herpes simplex. Vasculitis. Trauma, compression. Diabetes. Postinfectious or inflammatory.

MUSCLE WEAKNESS ICD-9CM # 728.9 Physical deconditioning. Impaired cardiac output (e.g., mitral stenosis, mitral regurgitation). Uremia, liver failure. Electrolyte abnormalities (hypokalemia, hyperkalemia, hypophosphatemia, hypercalcemia), hypoglycemia. Drug-induced (e.g., statin myopathy). Muscular dystrophies. Steroid myopathy. Alcoholic myopathy. Myasthenia gravis, Lambert-Eaton syndrome. Infections (polio, botulism, HIV, hepatitis, diphtheria, tick paralysis, neurosyphilis, brucellosis, TB, trichinosis). Pernicious anemia, other anemias, beriberi. Psychiatric illness (depression, somatization syndrome). Organophosphate or arsenic poisoning. Inflammatory myopathies (e.g., collagen vascular disease, RA, sarcoidosis). Endocrinopathies (e.g., adrenal insufficiency, hypothyroidism), diabetic neuropathy. Other: motor neuron disease, mitochondrial myopathy, rhabdomyolysis.

MUSCLE WEAKNESS, LOWER MOTOR NEURON VERSUS UPPER MOTOR NEURON23 ICD-9CM # 728.9

LOWER MOTOR NEURON Weakness, usually severe. Marked muscle atrophy. Fasciculations. Decreased muscle stretch reflexes. Clonus not present. Flaccidity. No Babinski’s sign.

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Myocardial Ischemia Asymmetric and may involve one limb only in the beginning to become generalized as the disease progresses.

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Neck Pain

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NAUSEA AND VOMITING ICD-9CM # 787.01

UPPER MOTOR NEURON Weakness, usually less severe. Minimal disuse muscle atrophy. No fasciculations. Increased muscle stretch reflexes. Clonus may be present. Spasticity. Babinski’s sign. Often initial impairment of only skilled movements. In the limbs the following muscles may be the only ones weak or weaker than the others: triceps; wrist and finger extensors; interossei; iliopsoas; hamstrings; and foot dorsiflexors, inverters and extroverters.

ICD-9CM # 414.8 ISCHEMIA (CHRONIC) 411.89 ISCHEMIA, ACUTE WITHOUT MI

NECK MASS16 ICD-9CM # 784.2

CONGENITAL ANOMALIES Thyroglossal duct cyst. Bronchial apparatus anomalies. Teratomas. Ranula. Dermoid cysts. Hemangioma. Laryngoceles. Cystic hygroma.

NONNEOPLASTIC INFLAMMATORY ETIOLOGIES Folliculitis. Adenopathy secondary to peritonsillar abscess. Retropharyngeal or parapharyngeal abscess. Salivary gland infections. Viral infections (mononucleosis, HIV, CMV). TB. Cat-scratch disease. Toxoplasmosis. Actinomyces. Atypical mycobacterium. Jugular vein thrombus.

NEOPLASM (PRIMARY OR METASTATIC) Lipoma.

NECK PAIN16 ICD-9CM # 723.1 NECK PAIN (NONDISCOGENIC) 959.09 NECK INJURY

INFLAMMATORY DISEASES Rheumatoid arthritis (RA). Spondyloarthropathies.

NONINFLAMMATORY DISEASE Cervical osteoarthritis. Diskogenic neck pain.

DIFFERENTIAL DIAGNOSIS

Atherosclerotic obstructive coronary artery disease. Nonatherosclerotic coronary artery disease: Coronary artery spasm. Congenital coronary artery anomalies: -Anomalous origin of coronary artery from pulmonary artery. -Aberrant origin of coronary artery from aorta or another coronary artery. -Coronary arteriovenous fistula. -Coronary artery aneurysm. Acquired disorders of coronary arteries: Coronary artery embolism. Dissection: -Surgical. -During percutaneous coronary angioplasty. -Aortic dissection. -Spontaneous. Extrinsic compression: -Tumors. -Granulomas. -Amyloidosis. Collagen-vascular disease: -Polyarteritis nodosa. -Temporal arteritis. -Rheumatoid arthritis. -Systemic lupus erythematosus. -Scleroderma. Miscellaneous disorders: -Irradiation. -Trauma. -Kawasaki’s disease. Syphilis. Hereditary disorders: Pseudoxanthoma elasticum. Gargoylism. Progeria. Homocystinuria. Primary oxaluria. “Functional” causes of myocardial ischemia in absence of anatomic coronary artery disease: Syndrome X. Hypertrophic cardiomyopathy. Dilated cardiomyopathy. Muscle bridge. Hypertensive heart disease. Pulmonary hypertension. Valvular heart disease, aortic stenosis, aortic regurgitation.

Section II

MYOCARDIAL ISCHEMIA22

Infections (viral, bacterial). Intestinal obstruction. Metabolic (uremia, electrolyte abnormalities, DKA, acidosis, etc.). Severe pain. Anxiety, fear. Psychiatric disorders (bulimia, anorexia nervosa). Medications (NSAIDs, erythromycin, morphine, codeine, aminophylline, chemotherapeutic agents, etc.). Withdrawal from substance abuse (drugs, alcohol). Head trauma. Vestibular or middle ear disease. Migraine headache. CNS neoplasms. Radiation sickness. PUD. Carcinoma of GI tract. Reye’s syndrome. Eye disorders. Abdominal trauma.

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Neurogenic Bladder Diffuse idiopathic skeletal hyperostosis. Fibromyalgia or myofascial pain.

INFECTIOUS CAUSES Meningitis. Osteomyelitis. Infectious diskitis.

NEOPLASMS Primary. Metastatic.

REFERRED PAIN Temporomandibular joint pain. Cardiac pain. Diaphragmatic irritation. Gastrointestinal sources (gastric ulcer, gallbladder, pancreas).

NEUROGENIC BLADDER17 ICD-9CM # 396.54

SUPRATENTORIAL CVA. Parkinson’s disease. Alzheimer’s disease. Cerebral palsy.

SPINAL CORD Spinal cord injury. Spinal stenosis. Central cord syndrome. ALS. Multiple sclerosis. Myelodysplasia.

PERIPHERAL NEUROPATHY Diabetes. Alcohol. Shingles. Syphilis.

NEUROLOGIC DEFICIT, FOCAL14 ICD-9CM # 436 CVA 435.9 TIA

TRAUMATIC: INTRACRANIAL, INTRASPINAL Subdural hematoma. Intraparenchymal hemorrhage. Epidural hematoma. Traumatic hemorrhagic necrosis.

INFECTIOUS Brain abscess. Epidural and subdural abscesses. Meningitis.

NEOPLASTIC Primary central nervous system tumors. Metastatic tumors. Syringomyelia. Vascular. Thrombosis. Embolism. Spontaneous hemorrhage: arteriovenous malformation, aneurysm, hypertensive.

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Nystagmus METABOLIC Hypoglycemia. B12 deficiency. Postseizure. Hyperosmolar nonketotic.

OTHER Migraine. Bell’s palsy. Psychogenic.

NEUROLOGIC DEFICIT, MULTIFOCAL14 ICD-9CM # 436 CVA 435.9 TIA Acute disseminated encephalomyelitis: postviral or postimmunization. Infectious encephalomyelitis: poliovirus, enteroviruses, arbovirus, herpes zoster, Epstein-Barr virus. Granulomatous encephalomyelitis: sarcoid. Autoimmune: systemic lupus erythematosus. Other: familial spinocerebellar degenerations.

NEUROPATHIES, PAINFUL23 ICD-9CM # 355.9 NEUROPATHY NOS 357.5 ALCOHOLIC 357.8 CHRONIC PROGRESSIVE OR RELAPSING 356.2 CONGENITAL SENSORY 356.0 DEJERINE-SOTTAS 356.60 DIABETIC POLYNEUROPATHY, TYPE II 356.61 DIABETIC POLYNEUROPATHY, TYPE I

MONONEUROPATHIES Compressive neuropathy (carpal tunnel, meralgia paresthetica). Trigeminal neuralgia. Ischemic neuropathy. Polyarteritis nodosa. Diabetic mononeuropathy. Herpes zoster. Idiopathic and familial brachial plexopathy.

POLYNEUROPATHIES Diabetes mellitus. Paraneoplastic sensory neuropathy. Nutritional neuropathy. Multiple myeloma. Amyloid. Dominantly inherited sensory neuropathy. Toxic (arsenic, thallium, metronidazole). AIDS-associated neuropathy. Tangier’s disease. Fabry’s disease.

NYSTAGMUS ICD-9CM # 379.50 NYSTAGMUS NOS 386.11 BENIGN POSITIONAL 386.2 CENTRAL POSITIONAL 379.59 CONGENITAL Medications (meperidine, barbiturates, phenytoin, phenothiazines, etc.). Multiple sclerosis. Congenital. Neoplasm (cerebellar, brainstem, cerebral). Labyrinthine or vestibular lesions.

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Ophthalmoplegia CNS infections. Optic atrophy. Other: Arnold-Chiari malformation, syringobulbia, chorioretinitis, meningeal cysts.

OPHTHALMOPLEGIA1 ICD-9CM # 378.9 OPHTHALMOPLEGIA NOS 378.52 CEREBELLAR ATAXIA SYNDROME 376.22 EXOPHTHALMIC

BILATERAL

UNILATERAL

PALPITATIONS19 ICD-9CM # 785.1 PALPITATIONS Anxiety. Electrolyte abnormalities (hypokalemia, hypomagnesemia). Exercise. Hyperthyroidism. Ischemic heart disease. Ingestion of stimulant drugs (cocaine, amphetamines, caffeine). Medications (digoxin, -blockers, calcium channel antagonists, hydralazines, diuretics, minoxidil). Hypoglycemia in type 1 DM. Mitral valve prolapse. Wolff-Parkinson-White (WPW) syndrome. Sick sinus syndrome.

PANCYTOPENIA22 ICD-9CM # 284.8

PANCYTOPENIA WITH HYPOCELLULAR BONE MARROW Acquired aplastic anemia. Constitutional aplastic anemia. Exposure to chemical or physical agents, including ionizing irradiation and chemotherapeutic agents. Some hematologic malignancies, including myelodysplasia and aleukemic leukemia.

PANCYTOPENIA WITH NORMAL OR INCREASED CELLULARITY OF HEMATOPOIETIC ORIGIN Some hematologic malignancies, including myelodysplasia, and some leukemias, lymphomas, and myelomas. Paroxysmal nocturnal hemoglobinuria. Hypersplenism. Vitamin B12, folate deficiencies. Overwhelming infection.

PANCYTOPENIA WITH BONE MARROW REPLACEMENT Tumor metastatic to marrow. Metabolic storage diseases. Osteopetrosis. Myelofibrosis.

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PARESTHESIAS ICD-9CM # 782.0 Multiple sclerosis. Nutritional deficiencies (thiamin, vitamin B12, folic acid). Compression of spinal cord or peripheral nerves. Medications (e.g., INH, lithium, nitrofurantoin, gold, cisplatin, hydralazine, amitriptyline, sulfonamides, amiodarone, metronidazole, dapsone, disulfiram, chloramphenicol). Toxic chemicals (e.g., lead, arsenic, cyanide, mercury, organophosphates). DM. Myxedema. Alcohol. Sarcoidosis. Neoplasms. Infections (HIV, Lyme disease, herpes zoster, leprosy, diphtheria). Charcot-Marie-Tooth syndrome and other hereditary neuropathies. Guillain-Barré neuropathy.

PELVIC MASS ICD-9CM # 789.39 Hemorrhagic ovarian cyst. Simple ovarian cyst (follicle or corpus luteum). Ovarian carcinoma, carcinoma of fallopian tube, colorectal carcinoma, metastatic carcinoma, prostate carcinoma, bladder carcinoma, lymphoma, Hodgkin’s disease. Cystadenoma, teratoma. Leiomyoma. Leiomyosarcoma. Diverticulitis, diverticular abscess. Appendiceal abscess, tuboovarian abscess. Paraovarian cyst. Hydrosalpinx.

PERICARDIAL EFFUSION ICD-9CM # 420.90 Pericarditis. Uremia. Myxedema. Neoplasm (leukemia, lymphoma, metastatic). Hemorrhage (trauma, leakage of thoracic aneurysm). SLE, rheumatoid disease. Myocardial infarction.

PLEURAL EFFUSIONS ICD-9CM # 511.9 PLEURAL EFFUSION, UNSPECIFIED

EXUDATIVE Neoplasm: bronchogenic carcinoma, breast carcinoma, mesothelioma, lymphoma, ovarian carcinoma, multiple myeloma, leukemia, Meigs’ syndrome. Infections: viral pneumonia, bacterial pneumonia, Mycoplasma, TB, fungal and parasitic diseases, extension from subphrenic abscess. Trauma. Collagen vascular diseases: SLE, RA, scleroderma, polyarteritis, Wegener’s granulomatosis. Pulmonary infarction. Pancreatitis. Postcardiotomy/Dressler’s syndrome. Drug-induced lupus erythematosus (hydralazine, procainamide). Postabdominal surgery.

DIFFERENTIAL DIAGNOSIS

Carotid-posterior (3rd cranial nerve, pupil involved communicating aneurysm). Diabetic-idiopathic (3rd or 6th cranial nerve, pupil spared). Myasthenia gravis. Brainstem stroke.

Pleural Effusions

Section II

Botulism. Myasthenia gravis. Wernicke’s encephalopathy. Acute cranial polyneuropathy. Brainstem stroke.

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Polyneuropathy

Polyneuropathy, Symmetric

Ruptured esophagus. Chronic effusion secondary to congestive failure.

ddC (dideoxycytidine). ddI (dideoxyinosine).

TRANSUDATIVE

DRUGS IN CARDIOLOGY

CHF. Hepatic cirrhosis. Nephrotic syndrome. Hypoproteinemia from any cause. Meigs’ syndrome.

Hydralazine. Perhexiline maleate. Procainamide. Disopyramide.

POLYNEUROPATHY

23

ICD-9CM # 357.9

PREDOMINANTLY MOTOR Guillain-Barré syndrome. Porphyria. Diphtheria. Lead. Hereditary sensorimotor neuropathy, types I and II. Paraneoplastic neuropathy.

PREDOMINANTLY SENSORY Diabetes. Amyloidosis. Leprosy. Lyme disease. Paraneoplastic neuropathy. Vitamin B12 deficiency. Hereditary sensory neuropathy, types I-IV.

PREDOMINANTLY AUTONOMIC Diabetes. Amyloidosis. Alcoholic neuropathy. Familial dysautonomias.

MIXED SENSORIMOTOR Systemic diseases: renal failure, hypothyroidism, acromegaly, rheumatoid arthritis, periarteritis nodosa, systemic lupus erythematosus, multiple myeloma, macroglobulinemia, remote effect of malignancy. Medications: isoniazid, nitrofurantoin, ethambutol, chloramphenicol, chloroquine, vincristine, vinblastine, dapsone, disulfiram, diphenylhydantoin, cisplatin, L-tryptophan. Environmental toxins: N-hexane, methyl N-butyl ketone, acrylamide, carbon disulfide, carbon monoxide, hexachlorophene, organophosphates. Deficiency disorders: malabsorption, alcoholism, vitamin B1 deficiency, Refsum’s disease, metachromatic leukodystrophy.

POLYNEUROPATHY, DRUG-INDUCED23 ICD-9CM # 357.6

DRUGS IN ONCOLOGY Vincristine. Procarbazine. Cisplatin. Misonidazole. Metronidazole (Flagyl). Taxol.

DRUGS IN INFECTIOUS DISEASES Isoniazid. Nitrofurantoin. Dapsone.

DRUGS IN RHEUMATOLOGY Gold salts. Chloroquine.

DRUGS IN NEUROLOGY AND PSYCHIATRY Diphenylhydantoin. Glutethimide. Methaqualone.

MISCELLANEOUS Disulfiram (Antabuse). Vitamin: pyridoxine (megadoses).

POLYNEUROPATHY, SYMMETRIC23 ICD-9CM # 357.9

ACQUIRED NEUROPATHIES Toxic: Drugs. Industrial toxins. Heavy metals. Abused substances. Metabolic/endocrine: Diabetes. Chronic renal failure. Hypothyroidism. Polyneuropathy of critical illness. Nutritional deficiency: Vitamin B12 deficiency. Alcoholism. Vitamin E deficiency. Paraneoplastic: Carcinoma. Lymphoma. Plasma cell dyscrasia: Myeloma, typical, atypical, and solitary forms. Primary systemic amyloidosis. Idiopathic chronic inflammatory demyelinating polyneuropathies. Polyneuropathies associated with peripheral nerve autoantibodies. Acquired immunodeficiency syndrome.

INHERITED NEUROPATHIES Neuropathies with biochemical markers Refsum’s disease. Bassen-Kornzweig disease. Tangier’s disease. Metachromatic leukodystrophy. Krabbe’s disease. Adrenomyeloneuropathy. Fabry’s disease. Neuropathies without biochemical markers or systemic involvement Hereditary motor neuropathy. Hereditary sensory neuropathy. Hereditary sensorimotor neuropathy.

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Polyuria

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QT Interval Prolongation

POLYURIA

PULMONARY LESIONS

ICD-9CM # 788.42

ICD-9CM # 518.3 PULMONARY INFILTRATE 518.89 PULMONARY NODULE 508.9 PULMONARY DISORDER DUE TO UNSPECIFIED EXTERNAL AGENT 861.20 PULMONARY INJURY NOS

DM. Diabetes insipidus. Primary polydipsia (compulsive water drinking). Hypercalcemia. Hypokalemia. Postobstructive uropathy. Diuretic phase of renal failure. Drugs: diuretics, caffeine, alcohol, lithium. Sickle cell trait or disease, chronic pyelonephritis (failure to concentrate urine). Anxiety, cold weather.

ICD-9CM # 572.3

INCREASED RESISTANCE TO FLOW

INCREASED PORTAL BLOOD FLOW Splenomegaly not caused by liver disease. Arterioportal fistula.

POSTMENOPAUSAL VAGINAL BLEEDING

PULMONARY NODULE, SOLITARY ICD-9CM # 518.89 Bronchogenic carcinoma. Granuloma from histoplasmosis. TB granuloma. Granuloma from coccidioidomycosis. Metastatic carcinoma. Bronchial adenoma. Bronchogenic cyst. Hamartoma. AV malformation. Other: fibroma, intrapulmonary lymph node, sclerosing hemangioma, bronchopulmonary sequestration.

ICD-9CM # 627.1 Hormone replacement therapy. Neoplasm (uterine, ovarian, cervical, vaginal, vulvar). Atrophic vaginitis. Vaginal infection. Polyp. Extragenital (GI, urinary). Tamoxifen. Trauma.

PRURITUS ICD-9CM # 698.9 PRURITUS NOS 697.0 PRURITUS ANI 698.1 PRURITUS, GENITAL ORGANS Dry skin. Drug-induced eruption, fiberglass exposure. Scabies. Skin diseases. Myeloproliferative disorders: mycosis fungoides, Hodgkin’s lymphoma, multiple myeloma, polycythemia vera. Cholestatic liver disease. Endocrine disorders: DM, thyroid disease, carcinoid. Carcinoma: breast, lung, gastric. Chronic renal failure. Iron deficiency. AIDS. Neurosis. Sjögren’s syndrome.

PURPURA ICD-9CM # 287.2 287.0 287.0 287.3 446.6

PURPURA NOS AUTOIMMUNE HENOCH-SCHÖNLEIN IDIOPATHIC THROMBOCYTOPENIC THROMBOCYTOPENIC

THROMBOTIC Trauma. Septic emboli, atheromatous emboli. DIC. Thrombocytopenia. Meningococcemia. Rocky Mountain spotted fever. Hemolytic-uremic syndrome. Viral infection: echo, coxsackie. Scurvy. Other: left atrial myxoma, cryoglobulinemia, vasculitis, hyperglobulinemic purpura.

QT INTERVAL PROLONGATION12 ICD-9CM # 794.31 Drugs: Class I antiarrhythmics (e.g., disopyramide, procainamide, quinidine). Class III antiarrhythmics. Tricyclic antidepressants. Phenothiazines.

DIFFERENTIAL DIAGNOSIS

Presinusoidal Portal or splenic vein occlusion (thrombosis, tumor). Schistosomiasis. Sarcoidosis. Sinusoidal Cirrhosis (all causes). Alcoholic hepatitis. Postsinusoidal Venoocclusive disease. Budd-Chiari syndrome. Constrictive pericarditis.

Section II

PORTAL HYPERTENSION1

TB. Legionella pneumophila. Mycoplasma pneumoniae. Viral pneumonia. Pneumocystis carinii. Hypersensitivity pneumonitis. Aspiration pneumonia. Fungal disease (aspergillosis, histoplasmosis). ARDS associated with pneumonia. Psittacosis. Sarcoidosis. Septic emboli. Metastatic cancer. Multiple pulmonary emboli. Rheumatoid nodules.

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Rectal Pain Astemizole. Terfenadine. Adenosine. Antibiotics (e.g., erythromycin and other macrolides). Antifungal agents. Pentamidine, chloroquine. Ischemic heart disease. Cerebrovascular disease. Rheumatic fever. Myocarditis. Mitral valve prolapse. Electrolyte abnormalities. Hypocalcemia. Hypothyroidism. Liquid protein diets. Organophosphate insecticides. Congenital prolonged QT syndrome.

RECTAL PAIN ICD-9CM # 569.42 Anal fissure. Thrombosed hemorrhoid. Anorectal abscess. Foreign bodies. Fecal impaction. Neoplasms (primary or metastatic). Pelvic inflammatory disease. Inflammation of sacral nerves. Compression of sacral nerves. Prostatitis. Other: proctalgia fugax, uterine abnormalities, myopathies, coccygodynia.

RED EYE ICD-9CM # 379.93 Infectious conjunctivitis (bacterial, viral). Allergic conjunctivitis. Acute glaucoma. Keratitis (bacterial, viral). Iritis. Trauma.

RENAL FAILURE, INTRINSIC OR PARENCHYMAL CAUSES22 ICD-9CM # 584. ACUTE, USE 4TH DIGIT .5 WITH ACUTE TUBULAR NECROSIS .6 WITH CORTICAL NECROSIS .7 WITH MEDULLARY NECROSIS .8 WITH OTHER UNSPECIFIED PATHOLOGIC CONDITION IN KIDNEY .9 RENAL FAILURE UNSPECIFIED 585 RENAL FAILURE, CHRONIC

ABNORMALITIES OF THE VASCULATURE Renal arteries: atherosclerosis, thromboembolism, arteritis. Renal veins: thrombosis. Microvasculature: vasculitis, thrombotic microangiopathy.

ABNORMALITIES OF GLOMERULI (ACUTE GLOMERULONEPHRITIS) Antiglomerular membrane disease (Goodpasture’s syndrome). Immune complex glomerulonephritis: SLE, postinfectious, idiopathic, membranoproliferative.

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Respiratory Failure, Hypoventilatory ABNORMALITIES OF INTERSTITIUM (ACUTE INTERSTITIAL NEPHRITIS) Drugs (e.g., antibiotics, NSAIDs, diuretics, anticonvulsants, allopurinol). Infectious pyelonephritis. Infiltrative: lymphoma, leukemia, sarcoidosis.

ABNORMALITIES OF TUBULES Physical obstruction (uric acid, oxalate, light chains). Acute tubular necrosis: Ischemic. Toxic (antibiotics, chemotherapy, immunosuppressives, radiocontrast dyes, heavy metals, myoglobin, hemolysed RBCs).

RENAL FAILURE, POSTRENAL CAUSES22 ICD-9CM # 584. ACUTE, USE 4TH DIGIT .5 WITH ACUTE TUBULAR NECROSIS .6 WITH CORTICAL NECROSIS .7 WITH MEDULLARY NECROSIS .8 WITH OTHER UNSPECIFIED PATHOLOGIC CONDITION IN KIDNEY .9 RENAL FAILURE UNSPECIFIED 585 RENAL FAILURE, CHRONIC

RENAL FAILURE, PRERENAL CAUSES22 ICD-9CM # 584. ACUTE, USE 4TH DIGIT .5 WITH ACUTE TUBULAR NECROSIS .6 WITH CORTICAL NECROSIS .7 WITH MEDULLARY NECROSIS .8 WITH OTHER UNSPECIFIED PATHOLOGIC CONDITION IN KIDNEY .9 RENAL FAILURE UNSPECIFIED 585 RENAL FAILURE, CHRONIC

DECREASED CARDIAC OUTPUT CHF. Arrhythmias. Pericardial constriction or tamponade. Pulmonary embolism.

HYPOVOLEMIA GI tract loss (vomiting, diarrhea, nasogastric suction). Blood losses (trauma, GI tract surgery). Renal losses (diuretics, mineralocorticoid deficiency, postobstructive diuresis). Skin losses (burns).

VOLUME REDISTRIBUTION (DECREASE IN EFFECTIVE BLOOD VOLUME) Hypoalbuminemic states (cirrhosis, nephrosis). Sequestration of fluid in “third” space (ischemic bowel, peritonitis, pancreatitis). Peripheral vasodilation (sepsis, vasodilators, anaphylaxis).

ALTERED RENAL VASCULAR RESISTANCE Increase in afferent vascular resistance (NSAIDs, liver disease, sepsis, hypercalcemia, cyclosporine). Decrease in efferent arteriolar tone (ACE inhibitors).

RESPIRATORY FAILURE, HYPOVENTILATORY16 ICD-9CM # 518.81 RESPIRATORY FAILURE

ABNORMAL RESPIRATORY CAPACITY (NORMAL RESPIRATORY WORKLOADS) Acute depression of central nervous system: Various causes.

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Scrotal Pain

ABNORMAL PULMONARY WORKLOADS

ABNORMAL EXTRAPULMONARY WORKLOADS Chronic thoracic cage disorders: Severe kyphoscoliosis. After thoracoplasty. After thoracic cage injury. Acute thoracic cage trauma and burns. Pneumothorax. Pleural fibrosis and effusions. Abdominal processes.

Neoplasm. Acute epididymitis. Orchitis. Trauma. Hernia. Torsion of spermatic cord. Torsion of epididymis. Torsion of testis. Insect bite. Folliculitis. Sebaceous cyst. Thrombosis of spermatic vein. Other: lymphedema, dermatitis, fat necrosis, Henoch-Schönlein purpura, idiopathic scrotal edema.

SEIZURE ICD-9CM # 780.39 Syncope. Alcohol abuse/withdrawal. TIA. Hemiparetic migraine. Psychiatric disorders. Carotid sinus hypersensitivity. Hyperventilation, prolonged breath holding. Hypoglycemia. Narcolepsy. Movement disorders (tics, hemiballismus). Hyponatremia. Brain tumor (primary or metastatic). Tetanus. Strychnine, phencyclidine poisoning.

SEXUALLY TRANSMITTED DISEASES, ANORECTAL REGION14 ICD-9CM # 569.49 INFECTION AND REGION

ULCERATIVE

SCROTAL PAIN16 ICD-9CM # 878.2 SCROTAL INJURY, TRAUMATIC 608.9 SCROTAL DISORDER NOS 608.4 SCROTAL CELLULITIS 608.83 SCROTAL HEMORRHAGE, NONTRAUMATIC 608.4 SCROTAL NODULE, INFLAMMATORY Torsion: Appendages. Spermatic cord. Infection: Orchitis. Abscess. Epididymitis. Neoplasia: Benign. Malignant. Incarcerated hernia. Trauma. Hydrocele. Spermatocele. Varicocele.

SCROTAL SWELLING ICD-9CM # 608.86 Hydrocele. Varicocele.

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Lymphogranuloma venereum. Herpes simplex virus. Early (primary) syphilis. Chancroid (Haemophilus ducreyi). Cytomegalovirus. Idiopathic (usually HIV positive).

NONULCERATIVE Condyloma acuminatum. Gonorrhea. Chlamydia (Chlamydia trachomatis). Syphilis.

SHOULDER PAIN ICD-9CM # 952.2 SHOULDER INJURY 718.81 SHOULDER INSTABILITY 726.19 SHOULDER LIGAMENT OR MUSCLE INSTABILITY 840.9 SHOULDER STRAIN, SITE UNSPECIFIED

WITH LOCAL FINDINGS IN SHOULDER Trauma: contusion, fracture, muscle strain, trauma to spinal cord. Arthrosis, arthritis, RA, ankylosing spondylitis. Bursitis, synovitis, tendinitis, tenosynovitis. Aseptic (avascular) necrosis. Local infection: septic arthritis, osteomyelitis, abscess, herpes zoster, TB.

DIFFERENTIAL DIAGNOSIS

Chronic obstructive pulmonary disease: Chronic bronchitis. Asthmatic bronchitis. Emphysema. Asthma and acute bronchial hyperreactivity syndromes. Upper airway obstruction. Interstitial lung diseases.

Shoulder Pain

Section II

Chronic central hypoventilation syndromes: Obesity-hypoventilation syndrome. Sleep apnea syndrome. Hypothyroidism. Shy-Drager syndrome (multisystem atrophy syndrome). Acute toxic paralysis syndromes: Botulism. Tetanus. Toxic ingestion or bites. Organophosphate poisoning. Neuromuscular disorders (acute and chronic): Myasthenia gravis. Guillain-Barré syndrome. Drugs. Amyotrophic lateral sclerosis. Muscular dystrophies. Polymyositis. Spinal cord injury. Traumatic phrenic nerve paralysis.

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Shoulder Pain by Location

Tardive Dyskinesia

WITHOUT LOCAL FINDINGS IN SHOULDER

INTRALUMINAL

Cardiovascular disorders: ischemic heart disease, pericarditis, aortic aneurysm. Subdiaphragmatic abscess, liver abscess. Cholelithiasis, cholecystitis. Pulmonary lesions: apical bronchial carcinoma, pleurisy, pneumothorax, pneumonia. GI lesions: PUD, gastric neoplasm, peptic esophagitis. Pancreatic lesions: carcinoma, calculi, pancreatitis. CNS abnormalities: neoplasm, vascular abnormalities. Multiple sclerosis. Syringomyelia. Polymyositis/dermatomyositis. Psychogenic. Polymyalgia rheumatica.

Foreign body. Gallstones. Bezoars. Barium. Ascaris infestation.

SHOULDER PAIN BY LOCATION ICD-9CM # 952.2 SHOULDER INJURY 726.19 SHOULDER LIGAMENT OR MUSCLE INSTABILITY 840.8 SHOULDER SEPARATION

TOP OF SHOULDER (C4) Cervical source. Acromioclavicular. Sternoclavicular. Diaphragmatic.

SPLENOMEGALY ICD-9CM # 789.2 SPLENOMEGALY UNSPECIFIED 789.51 CHRONIC CONGESTIVE 789.2 UNKNOWN ORIGIN Hepatic cirrhosis. Neoplastic involvement: CML, CLL, lymphoma, multiple myeloma. Bacterial infections: TB, infectious endocarditis, typhoid fever, splenic abscess. Viral infections: infectious mononucleosis, viral hepatitis, HIV. Gaucher’s disease and other lipid storage diseases. Sarcoidosis. Parasitic infections (malaria, kala-azar, histoplasmosis). Hereditary and acquired hemolytic anemias. Idiopathic thrombocytopenic purpura (ITP). Collagen vascular disorders: SLE, RA (Felty’s syndrome), polyarteritis nodosa. Serum sickness, drug hypersensitivity reaction. Splenic cysts and benign tumors: hemangioma, lymphangioma. Thrombosis of splenic or portal vein. Polycythemia vera, myeloid metaplasia.

SUPEROLATERAL (C5)

STEATOHEPATITIS

Rotator cuff tendinitis. Impingement. Adhesive capsulitis. Glenohumeral arthritis.

ICD-9CM # 571.8 Alcohol abuse. Obesity. Diabetes mellitus. Parenteral nutrition. Medications (high-dose estrogen, amiodarone, corticosteroids, methotrexate, nifedipine). Jejunoileal bypass. Abetalipoproteinemia. Wilson’s disease.

ANTERIOR Bicipital tendinitis and rupture. Glenoid labral tear. Adhesive capsulitis. Glenohumeral arthritis. Osteonecrosis.

AXILLARY Neoplasm (Pancoast’s, mediastinal). Herpes zoster.

SMALL BOWEL OBSTRUCTION

14

ICD-9CM # 751.1 SMALL INTESTINE OBSTRUCTION, CONGENITAL 560.81 SMALL INTESTINE OBSTRUCTION DUE TO ADHESION

INTRINSIC Inflammatory (Crohn’s, radiation enteritis). Neoplasms (metastatic or primary). Intussusception. Traumatic (hematoma).

EXTRINSIC Hernias (internal and external). Adhesions. Volvulus. Compressing masses (tumors, abscesses, hematomas).

STROKE22 ICD-9CM # 436 ACUTE STROKE Hypoglycemia. Drug overdose or intoxication. Hysterical conversion reaction. Hyperventilation. Metabolic encephalopathy. Migraine. Syncope. Transient global amnesia. Seizures. Vestibular vertigo.

TARDIVE DYSKINESIA16 ICD-9CM # 781.3 DYSKINESIA 300.11 HYSTERICAL DYSKINESIA 333.82 OROFACIAL DYSKINESIA 307.9 PSYCHOGENIC DYSKINESIA

DIFFERENTIAL DIAGNOSIS Medications (antidepressants, anticholinergics, amphetamines, antipsychotics, lithium, L-dopa, phenytoin). Brain neoplasms. Ill-fitting dentures.

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Taste and Smell Loss Huntington’s disease. Idiopathic dystonias (tics, blepharospasm, aging). Wilson’s disease. Extrapyramidal syndrome (postanoxic or postencephalitic). Torsion dystonia.

TASTE AND SMELL LOSS1 ICD-9CM # 781.1 SMELL AND TASTE DISTURBANCE OF SENSATION

TASTE

SMELL

TENDINOPATHY14 ICD-9CM # 727.9

INTRINSIC FACTORS Anatomic factors Malalignment. Muscle weakness or imbalance. Muscle inflexibility. Decreased vascularity. Systemic factors Inflammatory conditions (e.g., SLE). Quinolone-induced tendinopathy. Age-related factors Tendon degeneration. Increased tendon stiffness. Tendon calcification. Decreased vascularity.

EXTRINSIC FACTORS Repetitive mechanical load Excessive duration. Excessive frequency. Excessive intensity. Poor technique. Workplace factors. Equipment problems Footwear. Athletic field surface. Equipment factors (e.g., racquet size). Protective gear.

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THROMBOCYTOPENIA ICD-9CM # 287.3 CONGENITAL OR PRIMARY 287.4 SECONDARY 287.5 THROMBOCYTOPENIA NOS

INCREASED DESTRUCTION Immunologic Drugs: quinine, quinidine, digitalis, procainamide, thiazide diuretics, sulfonamides, phenytoin, aspirin, penicillin, heparin, gold, meprobamate, sulfa drugs, phenylbutazone, nonsteroidal antiinflammatory drugs (NSAIDs), methyldopa, cimetidine, furosemide, INH, cephalosporins, chlorpropamide, organic arsenicals, chloroquine, platelet glycoprotein IIb/IIIa receptor inhibitors, ranitidine, indomethacin, carboplatin, ticlopidine, clopidogrel. Idiopathic thrombocytopenic purpura (ITP). Transfusion reaction: transfusion of platelets with plasminogen activator (PLA) in recipients without PLA-1. Collagen vascular diseases (e.g., systemic lupus erythematosus [SLE]). Autoimmune hemolytic anemia. Lymphoreticular disorders (e.g., CLL). Nonimmunologic Prosthetic heart valves. Thrombotic thrombocytopenic purpura (TTP). Sepsis. DIC. Hemolytic-uremic syndrome (HUS). Giant cavernous hemangioma.

DECREASED PRODUCTION Abnormal marrow. Marrow infiltration (e.g., leukemia, lymphoma, fibrosis). Marrow suppression (e.g., chemotherapy, alcohol, radiation). Hereditary disorders. Wiskott-Aldrich syndrome: X-linked disorder characterized by thrombocytopenia, eczema, and repeated infections. May-Hegglin anomaly: increased megakaryocytes but ineffective thrombopoiesis. Vitamin deficiencies (e.g., vitamin B12, folic acid).

SPLENIC SEQUESTRATION, HYPERSPLENISM DILUTIONAL, AS A RESULT OF MASSIVE TRANSFUSION

THROMBOCYTOSIS ICD-9CM # 289.9 THROMBOCYTOSIS, ESSENTIAL Iron deficiency. Posthemorrhage. Neoplasms (GI tract). CML. Polycythemia vera. Myelofibrosis with myeloid metaplasia. Infections. After splenectomy. Hemophilia. Pancreatitis. Cirrhosis. Idiopathic.

DIFFERENTIAL DIAGNOSIS

Local: allergic rhinitis, sinusitis, nasal polyposis, bronchial asthma. Systemic: renal failure, hepatic failure, nutritional deficiency (vitamin B12), Cushing’s syndrome, hypothyroidism, DM, infection (viral hepatitis, influenza), drugs (nasal sprays, antibiotics). Neurologic: head trauma, multiple sclerosis, Parkinson’s disease, frontal brain tumor.

Thrombocytosis

Section II

Local: radiation therapy. Systemic: cancer, renal failure, hepatic failure, nutritional deficiency (vitamin B12, zinc), Cushing’s syndrome, hypothyroidism, DM, infection (influenza), drugs (antirheumatic and antiproliferative). Neurologic: Bell’s palsy, familial dysautonomia, multiple sclerosis.

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Tremor TREMOR ICD-9CM # 781.0 TREMOR NOS 333.1 BENIGN ESSENTIAL TREMOR 333.1 FAMILIAL TREMOR

TREMOR PRESENT AT REST Parkinsonism. CNS neoplasms. Tardive dyskinesia.

POSTURAL TREMOR (PRESENT DURING MAINTENANCE OF A POSTURE) Essential senile tremor.

ACTION TREMOR (PRESENT WITH MOVEMENT) Anxiety. Medications (bronchodilators, caffeine, corticosteroids, lithium, etc.). Endocrine disorders (hyperthyroidism, pheochromocytoma, carcinoid). Withdrawal from substance abuse.

URINARY RETENTION, ACUTE ICD-9CM # 788.20 Mechanical obstruction: urethral stone, foreign body, urethral stricture, BPH, prostate carcinoma, prostatitis, trauma with hematoma formation). Neurogenic bladder. Neurologic disease (MS, parkinsonism, tabes dorsalis, CVA). Spinal cord injury. CNS neoplasm (primary or metastatic). Spinal anesthesia. Lower urinary tract instrumentation. Medications (antihistamines, antidepressants, narcotics, anticholinergics). Abdominal or pelvic surgery. Alcohol toxicity. Anxiety. Encephalitis. Postoperative pain. Encephalitis. Spina bifida occulta.

URINE, RED17 ICD-9CM # CODE VARIES WITH SPECIFIC DIAGNOSIS

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Vertigo Phenytoin. Dyes Azo dyes. Eosin. Foods Beets, berries, maize. Rhodamine B. Metabolic Porphyrins. Serratia marcescens (red diaper syndrome). Urate crystalluria.

UROPATHY, OBSTRUCTIVE22 ICD-9CM # 599.6

INTRINSIC CAUSES Intraluminal Intratubular deposition of crystals (uric acid, sulfas). Stones. Papillary tissue. Blood clots. Intramural Functional. Ureter (ureteropelvic or ureterovesical dysfunction). Bladder (neurogenic): spinal cord defect or trauma, diabetes, multiple sclerosis, Parkinson’s disease, cerebrovascular accidents. Bladder neck dysfunction. Anatomic Tumors. Infection, granuloma. Strictures.

EXTRINSIC CAUSES Originating in the reproductive system Prostate: benign hypertrophy or cancer. Uterus: tumors, prolapse. Ovary: abscess, tumor, cysts. Originating in the vascular system Aneurysms (aorta, iliac vessels). Aberrant arteries (ureteropelvic junction). Venous (ovarian veins, retrocaval ureter). Originating in the gastrointestinal tract: Crohn’s disease, pancreatitis, appendicitis, tumors Originating in the retroperitoneal space Inflammations. Fibrosis. Tumor, hematomas.

WITH A POSITIVE DIPSTICK Hematuria. Hemoglobinuria: negative urinalysis. Myoglobinuria: negative urinalysis.

WITH A NEGATIVE DIPSTICK Drugs Aminosalicylic acid. Deferoxamine mesylate. Ibuprofen. Phenacetin. Phenolphthalein. Phensuximide. Rifampin. Anthraquinone laxatives. Doxorubicin. Methyldopa. Phenazopyridine. Phenothiazine.

VERTIGO ICD-9CM # 780.4 VERTIGO NOS 386.11 BENIGN PAROXYSMAL POSITIONAL 386.2 CENTRAL ORIGIN 386.10 PERIPHERAL 386.12 VESTIBULAR (NEURONITIS)

PERIPHERAL Otitis media. Acute labyrinthitis. Vestibular neuronitis. Benign positional vertigo. Meniere’s disease. Ototoxic drugs: streptomycin, gentamicin. Lesions of the eighth nerve: acoustic neuroma, meningioma, mononeuropathy, metastatic carcinoma. Mastoiditis.

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Vision Loss, Acute, Painful CNS OR SYSTEMIC Vertebrobasilar artery insufficiency. Posterior fossa tumor or other brain tumors. Infarction/hemorrhage of cerebral cortex, cerebellum, or brainstem. Basilar migraine. Metabolic: drugs, hypoxia, anemia, fever. Hypotension/severe hypertension. Multiple sclerosis. CNS infections: viral, bacterial. Temporal lobe epilepsy. Arnold-Chiari malformation, syringobulbia. Psychogenic: ventilation, hysteria.

VISION LOSS, ACUTE, PAINFUL

ICD-9CM # 368.11 VISION LOSS, SUDDEN Retinal artery occlusion. Optic neuritis. Retinal vein occlusion. Vitreous hemorrhage. Retinal detachment. Exudative macular degeneration. CVA. Ischemic optic neuropathy. Factitious. Somatization syndrome, anxiety reaction.

VISION LOSS, CHRONIC, PROGRESSIVE ICD-9CM # 369.9 VISION LOSS NOS Cataract. Macular degeneration. Cerebral neoplasm. Refractive error. Open-angle glaucoma.

VISION LOSS, MONOCULAR, TRANSIENT ICD-9CM # 369.9 Thromboembolism. Vasculitis. Migraine (vasospasm). Anxiety reaction. CNS tumor. Temporal arteritis. Multiple sclerosis.

VOLUME DEPLETION1 ICD-9CM # 276.5 Gastrointestinal losses: Upper: bleeding, nasogastric suction, vomiting. Lower: bleeding, diarrhea, enteric or pancreatic fistula, tube drainage.

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Renal losses: Salt and water: diuretics, osmotic diuresis, postobstructive diuresis, acute tubular necrosis (recovery phase), salt-losing nephropathy, adrenal insufficiency, renal tubular acidosis. Water loss: diabetes insipidus. Skin and respiratory losses: Sweat, burns, insensible losses. Sequestration without external fluid loss: Intestinal obstruction, peritonitis, pancreatitis, rhabdomyolysis, internal bleeding.

VOLUME EXCESS1 ICD-9CM # CODE VARIES WITH SPECIFIC DIAGNOSIS

PRIMARY RENAL SODIUM RETENTION (INCREASED EFFECTIVE CIRCULATING VOLUME) Renal failure, nephritic syndrome, acute glomerulonephritis. Primary hyperaldosteronism. Cushing syndrome. Liver disease.

SECONDARY RENAL SODIUM RETENTION (DECREASED EFFECTIVE CIRCULATING VOLUME) Heart failure. Liver disease. Nephrotic syndrome (minimal change disease).

VOMITING ICD-9CM # 787.03 GI disturbances: Obstruction: esophageal, pyloric, intestinal. Infections: viral or bacterial enteritis, viral hepatitis, food poisoning, gastroenteritis. Pancreatitis. Appendicitis. Biliary colic. Peritonitis. Perforated bowel. Diabetic gastroparesis. Other: gastritis, PUD, IBD, GI tract neoplasms. Drugs: morphine, digitalis, cytotoxic agents, bromocriptine. Severe pain: MI, renal colic. Metabolic disorders: uremia, acidosis/alkalosis, hyperglycemia, DKA, thyrotoxicosis. Trauma: blows to the testicles, epigastrium. Vertigo. Reye’s syndrome. Increased intracranial pressure. CNS disturbances: trauma, hemorrhage, infarction, neoplasm, infection, hypertensive encephalopathy, migraine. Radiation sickness. Motion sickness. Bulimia, anorexia nervosa. Psychogenic: emotional disturbances, offensive sights or smells. Severe coughing. Pyelonephritis. Boerhaave’s syndrome. Carbon monoxide poisoning.

DIFFERENTIAL DIAGNOSIS

VISION LOSS, ACUTE, PAINLESS

Vomiting

Section II

ICD-9CM # 368.11 VISION LOSS, SUDDEN Acute angle-closure glaucoma. Corneal ulcer. Uveitis. Endophthalmitis. Factitious. Somatization syndrome. Trauma.

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Vulvar Lesions VULVAR LESIONS5 ICD-9CM # 625.8 VULVAR MASS 098.0 VULVAR ULCER, GONOCOCCAL 091.0 VULVAR ULCER, SYPHILITIC 616.51 BEHÇET’S 624.0 LEUKOPLAKIA 624.8 DYSPLASIA 233.3 CARCINOMA 616.9 INFLAMMATORY LESION 624.4 VULVAR SCAR (OLD) 624.1 VULVAR ATROPHY

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Weight Gain Vitiligo. Partial albinism. Intertrigo. Radiation treatment.

DARK LESION Lentigo. Nevi (mole). Neoplasm (see Neoplasm, Vulvar, below). Reactive hyperpigmentation. Seborrheic keratosis. Pubic lice.

RED LESION

ULCERATIVE LESION

Infection/infestation Fungal infection: Candida. Tinea cruris. Intertrigo. Pityriasis versicolor. Sarcoptes scabiei. Erythrasma: Corynebacterium minutissimum. Granuloma inguinale: Calymmatobacterium granulomatis. Folliculitis: Staphylococcus aureus. Hidradenitis suppurativa. Behçet’s syndrome. Inflammation Reactive vulvitis. Chemical irritation: Detergent. Dyes. Perfume. Spermicide. Lubricants. Hygiene sprays. Podophyllum. Topical 5-FU. Saliva. Gentian violet. Semen. Mechanical trauma: scratching. Vestibular adenitis. Essential vulvodynia. Psoriasis. Seborrheic dermatitis. Neoplasm Vulvar intraepithelial neoplasia (VIN): Mild dysplasia. Moderate dysplasia. Severe dysplasia. Carcinoma-in-situ. Vulvar dystrophy. Bowen’s disease. Invasive cancer: Squamous cell carcinoma. Malignant melanoma. Sarcoma. Basal cell carcinoma. Adenocarcinoma. Paget’s disease. Undifferentiated.

Infection Herpes simplex. Vaccinia. Treponema pallidum. Granuloma inguinale. Pyoderma. Tuberculosis. Noninfection Behçet’s disease. Crohn’s disease. Pemphigus. Pemphigoid. Hidradenitis suppurativa (see Neoplasm, Vulvar, below). Neoplasm Basal cell carcinoma. Squamous cell carcinoma. Vulvar tumor 1 cm: Condyloma acuminatum. Molluscum contagiosum. Epidermal inclusion. Vestibular cyst. Mesenephric duct. VIN. Hemangioma. Hidradenoma. Neurofibroma. Syringoma. Accessory breast tissue. Acrochordon. Endometriosis. Fox-Fordyce disease. Pilonidal sinus. Vulvar tumor 1 cm: Bartholin cyst or abscess. Lymphogranuloma venereum. Fibroma. Lipoma. Verrucous carcinoma. Squamous cell carcinoma. Hernia. Edema. Hematoma. Acrochordon. Epidermal cysts. Neurofibromatosis. Accessory breast tissue.

WHITE LESION

WEIGHT GAIN

Vulvar dystrophy: Lichen sclerosus. Vulvar dystrophy. Vulvar hyperplasia. Mixed dystrophy. VIN.

ICD-9CM # 783.1 ABNORMAL WEIGHT GAIN 278.00 OBESITY Sedentary lifestyle. Fluid overload. Discontinuation of tobacco abuse.

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Weight Loss Endocrine disorders (hypothyroidism, hyperinsulinism associated with maturity-onset DM, Cushing’s syndrome, hypogonadism, insulinoma, hyperprolactinemia, acromegaly). Medications (nutritional supplements, antidepressants, glucocorticoids, etc.). Anxiety disorders with compulsive eating. Laurence-Moon-Biedl syndrome, Prader-Willi syndrome, other congenital diseases. Hypothalamic injury (rare; 100 cases reported in medical literature).

WEIGHT LOSS ICD-9CM # 783.2 ABNORMAL WEIGHT LOSS

ICD-9CM # 786.09 Asthma. COPD. Interstitial lung disease. Infections (pneumonia, bronchitis, bronchiolitis, epiglottitis). Cardiac asthma. GERD with aspiration. Foreign body aspiration. Pulmonary embolism. Anaphylaxis. Obstruction airway (neoplasm, goiter, edema or hemorrhage from trauma, aneurysm, strictures, spasm). Carcinoid syndrome.

XEROSTOMIA16 ICD-9CM # 527.7 Medications Tricyclic antidepressants: amitriptyline (Elavil), doxepin (Sinequan). Antihistamines: diphenhydramine (Benadryl), chlorpheniramine (Chlor-Trimeton), promethazine (Phenergan), and many cold and decongestant preparations.

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Anticholinergic agents: antiemetics such as scopolamine, antispasmodic agents such as oxybutynin chloride (Ditropan). Dehydration Debility. Fever. Polyuria Alcohol intake. Arrhythmia. Diabetes. Previous head and neck irradiation Systemic diseases Sjögren’s syndrome. Sarcoidosis. Amyloidosis.

REFERENCES 1. Andreoli TE, editor: Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders. 2. Barkin RM, Rosen P: Emergency pediatrics: a guide to ambulatory care, ed 5, St Louis, 1998, Mosby. 3. Behrman RE: Nelson textbook of pediatrics, ed 16, Philadelphia, 2000, WB Saunders. 4. Conn R: Current diagnosis, ed 9, Philadelphia, 1997, WB Saunders. 5. Danakas G, editor: Practical guide to the care of the gynecologic/ obstetric patient, St Louis, 1997, Mosby. 6. Goldberg RJ: The care of the psychiatric patient, ed 22, St Louis, 1998, Mosby. 7. Goldman L, Ausiello D: Cecil textbook of medicine, ed 21, Philadelphia, 2004, WB Saunders. 8. Goldman L, Braunwauld E, editors: Primary cardiology, Philadelphia, 1998, WB Saunders. 9. Gorbach SL: Infectious diseases, ed 2, Philadelphia, 1998, WB Saunders. 10. Harrington J: Consultation in internal medicine, ed 2, St Louis, 1997, Mosby. 11. Kassirer J, editor: Current therapy in adult medicine, ed 4, St Louis, 1998, Mosby. 12. Khan MG: Rapid ECG interpretation, Philadelphia, 2003, WB Saunders. 13. Klippel J, editor: Practical rheumatology, London, 1995, Mosby. 14. Marx J, editor: Rosen’s emergency medicine: concepts and clinical practice, ed 5, St Louis, 2002, Mosby. 15. Moore WT, Eastman RC: Diagnostic endocrinology, ed 2, St Louis, 1996, Mosby. 16. Noble J, editor: Primary care medicine, ed 3, St Louis, 2001, Mosby. 17. Nseyo UO: Urology for primary care physicians, Philadelphia, 1999, WB Saunders. 18. Rakel RE: Principles of family practice, ed 6, Philadelphia, 2002, WB Saunders. 19. Seller RH: Differential diagnosis of common complaints, ed 4, Philadelphia, 2000,WB Saunders. 20. Siedel HM, editor: Mosby’s guide to physical examination, ed 4, St Louis, 1999, Mosby. 21. Specht N: Practical guide to diagnostic imaging, St Louis, 1998, Mosby. 22. Stein JH, editor: Internal medicine, ed 5, St Louis, 1998, Mosby. 23. Wiederholt WC: Neurology for non-neurologists, ed 4, Philadelphia, 2000, WB Saunders.

DIFFERENTIAL DIAGNOSIS

WHEEZING

Xerostomia

Section II

Malignancy. Psychiatric disorders (depression, anorexia nervosa). New-onset DM. Malabsorption. COPD. AIDS. Uremia, liver disease. Thyrotoxicosis, pheochromocytoma, carcinoid syndrome. Addison’s disease. Intestinal parasites. Peptic ulcer disease. Inflammatory bowel disease. Food faddism. Postgastrectomy syndrome.

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Clinical Algorithms

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PLEASE NOTE: These algorithms are designed to assist clinicians in the evaluation and treatment of patients. They may not

apply to all patients with a particular condition and are not intended to replace a clinician’s individual judgment.

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ABUSE, GERIATRIC ICD-9CM # 995.81 • Assessment of general quality of care and relationships in household or institution • Assessment of patient on their own, including assessment of mental capacity • Assessment of suspected abuser or their problems • Liaison with other professionals wherever possible, subject to confidentiality • Full documentation

Victim is capable of making necessary decisions

Victim is not willing to accept help

Victim is not capable of making necessary decisions

Victim is willing to accept help

Victim is not willing to accept help

In conjunction with other professionals

• Establish victim's needs • Implement safety plan • Educate/provide information about abuse, rights, and local services • Provide services to victim, abuser, or both, that focus on preventing further abuse • Assist with legal interventions • Make sure that situation is monitored by someone

• Ensure protection for the victim either in terms of physical safety and/or proper financial arrangements • Provide relevant help to the abuser • Liaise with police if serious crime has been committed • Make sure that situation is monitored by someone

CLINICAL ALGORITHMS

FIGURE 3-1 Management of geriatric abuse. (From Tallis RC, Fillit HM [eds]: Brocklehurst’s textbook of geriatric medicine and gerontology, ed 6, London, 2003, Churchill Livingstone.)

Section III

• Educate/provide information about abuse, rights, and local services • Provide written information about getting help in an emergency • Assure victim of support and help if requested • Develop a safety plan • Develop a follow-up plan preferably to involve monitoring of the situation • Legal intervention may be necessary where a criminal offense has been committed, or the victim's life or health are in danger

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ACIDOSIS, METABOLIC ICD-9CM # 276.2 Metabolic acidosis suspected Na, K, Cl, HCO 3, anion gap PaO2, PaCO2, pH, base excess (deficit) Metabolic acidosis confirmed

pH 7.43

pH 7.39

Respiratory alkalosis

Metabolic acidemia

Treat hypocapnia

Anion gap 12 mEq/L

Drug history

Anion gap 12 mEq/L

No drug history

Stop drug Ketoacids in blood or urine

Glucose 200 mg/dl

Glucose 200 mg/dl

Diabetes mellitus

Suspect alcoholic ketoacidosis or starvation

IV fluid Insulin If pH 7.1, NaHCO3 IV

IV fluids Nutrition

No ketoacids in blood or urine

Creatinine 8 mg/dl

Creatinine 8 mg/dl

Renal failure

Predisposing factor for lactic acidosis

Consider: Dialysis If pH 7.1, NaHCO3 IV

FIGURE 3-2 Suspected metabolic acidosis. (From Greene HL, Johnson WP, Lemke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

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ACIDOSIS, METABOLIC—cont’d

L-lactate 2.5 mmol/L

L-lactate

2.5 mmol/L

D-lactate

0.0 mmol/L

Lactic acidosis

Treat underlying cause if pH 7.1, NaHCO3 IV

Abnormal gut flora

Nonabsorbable antibiotic

Drug

Excess fluid

Stop drug

Expansion acidosis

Treat underlying problem If pH 7.2, NaHCO3 IV

Carbonic anhydrase inhibitor

Clinical suspicion of aldosterone deficiency

Stop drug

Hyponatremia

Consider: Renal tubular acidosis

If pH 7.1, NaHCO3 IV Assess urine pH during acid load

FIGURE 3-2 (Continued) Suspected metabolic acidosis. (From Greene HL, Johnson WP, Lemke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Blood sample for cortisol level Hydrocortisone, 100 mg q6h

Section III

Observe Restrict fluid

Renal loss HCO 3

Small or large bowel fluid loss

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ALCOHOLISM ICD-9CM # 303.9 Step I: Ask about alcohol use Consumption Per week Per occasion CAGE questions (1 point for each yes answer): Have you ever felt that you should Cut down on your drinking? Have people Annoyed you by criticizing your drinking? Have you ever felt bad or Guilty about your drinking? Have you ever had a drink first thing in the morning to steady your nerves or to get rid of a hangover (Eye opener)? Men: 14 drinks/week or 4 per occasion Women: 7 drinks/week or 3 per occasion or CAGE score 1 Step II: Assess for alcohol-related problems At risk: May be alcohol-dependent: Drinking above recommended CAGE score 3 or 1 of the levels or in high-risk situations following: Personal or family history of Preoccupied with drinking alcohol-related problems Unable to stop once started Current alcohol-related problems: Drinking to avoid CAGE score 1-2 (in past year) withdrawal symptoms Evidence of alcohol-related Tolerance medical or behavioral problems

Step III: Advise appropriate action State medical concerns about drinking Agree on plan of action: At risk or current problems: Alcohol-dependent: Advise to cut down Advise to abstain Set specific drinking goal Refer to specialist

Step IV: Monitor patient progress All patients: Patients referred for alcohol treatment: Consider scheduling Review updates from separate follow-up treatment specialist visit or phone call Monitor for depression Review progress and and anxiety reinforce efforts at each follow-up visit FIGURE 3-3 Screening and brief intervention for alcohol problems in clinical practice. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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ALKALOSIS, METABOLIC ICD-9CM # 273.6 Metabolic alkalosis suspected Na, K, Cl, HCO 3, PaO2, PaCO2, pH, base excess (deficit) Metabolic alkalosis confirmed

pH 7.43

pH 7.39

Metabolic alkalemia

Respiratory acidemia

Hypoxemia

Treat cause of hypercapnia

No hypoxemia

Titrate FiO2

Urine Cl 10 mmol/L

Primary hypermineralocorticoid

Diuretic

Hypovolemia

Normal saline, KCI

IV KCI

Hypervolemia

Exogenous steroids

Stop drug

Renal failure plus alkali therapy

Cushing’s disease or primary aldosteronism

Hypovolemia

Consider: Dialysis

Treat underlying cause

Spironolactone, 25-100 mg PO Consider: K, Mg repletion, Acetazolamide 500 mg

Hypervolemia

Consider: IV HCl 0.2 mol/L

FIGURE 3-4 Suspected metabolic alkalosis. (From Greene HL, Johnson WP, Lemke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Stop drug

Severe K deficit

Section III

Vomiting, nasogastric suctioning

Urine Cl 15 mmol/L

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ANEMIA ICD-9CM # 285.9 Anemia NOS Anemia

Reticulocyte count

Low

Not elevated

Elevated

MCV

Hemolysis or blood loss

Normal

• Iron deficiency (severe) • Anemia of chronic disease (some cases) • Thalassemia trait (reticulocyte count may be elevated) • Sideroblastic anemias (some cases) • Lead poisoning (rare in adults)

• Bone marrow aplasia/hypoplasia • Renal insufficiency • Pure red cell aplasia • Myelofibrosis • Myelophthisis • Myelodysplasia (most cases) • Anemia of chronic disease (most cases) • Mixed microcytic and macrocytic anemias • Iron deficiency (mild to moderate) • Hemoglobinopathies with right-shifted oxygen dissociation curves (physiologic anemia)

High • Cobalamin (vitamin B12) deficiency • Folate deficiency • Treatment with drugs that interfere with DNA synthesis and cell division • Prior cancer chemotherapy • Myelodysplasia (some cases) • Hypothyroidism • Liver disease

No symptoms or signs of blood loss

History or physical examination findings of acute or chronic blood loss Blood loss

Hemolysis

Acquired

Inherited/congenital

• Immune hemolysis Autoimmune Drug-induced Alloimmune • Traumatic (microangiopathic and macroangiopathic) hemolysis TTP/HUS/HELLP DIC Vasculitis (rare cause) Eclampsia Malignant hypertension Prosthetic heart valves Arterial grafts • Hypersplenism • Membrane abnormalities Acanthocytes (spur cells) Echinocytes (burr cells) Paroxysmal nocturnal hemoglobinuria Thermal injury (burns) • Infection Malaria Babesiosis Bartonellosis Clostridia toxin • Osmotic damage Fresh water drowning

• RBC membranopathies Spherocytosis Elliptocytosis Pyropoikilocytosis Stomatocytosis • RBC enzymopathies G6PD deficiency Pyruvate kinase deficiency Other rarer deficiencies of enzymes of Embden Meyerhof pathway, hexose monophosphate shunt, or nucleotide metabolism • Hemoglobinopathies Thalassemias Hemoglobins S, C, D, E Unstable hemoglobins Other rarer hemoglobinopathies

FIGURE 3-5 Algorithm for diagnosis of anemias. DIC, Disseminated intravascular coagulation; HELLP, hepatomegaly-elevated liver (function tests)-low platelets; HUS, hemolytic-uremic syndrome; MCV, mean corpuscular volume; RBC, red blood cell; TTP, thrombotic thrombocytopenic purpura. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

Bone marrow aspiration and biopsy to evaluate for myelodysplasia

No improvement with discontinuing medication

No possible offending medication

Correct hypothyroidism

Abnormal thyroid function tests

No megaloblastic changes on peripheral blood smear

No treatment needed

Down syndrome

Reticulocyte count elevated

Examine peripheral smear for indices of nonreticulocyte red cells

Diagnosis: spurious elevation of MCV

FIGURE 3-6 Differential diagnosis of macrocytic anemia. (From Rakel RE [ed]: Principles of family practice, ed 6, Philadelphia, 2002, WB Saunders.)

Change medication

Improvement with discontinuing medication

Possible medication effect

Evaluate for causes of liver disease

Abnormal liver function tests

CLINICAL ALGORITHMS

Initiate B12 replacement

Evaluate for dietary causes

Diagnosis: folic acid deficiency

Diagnosis: B12 deficiency

Serum B12 normal or increased, folate normal

Reticulocyte count not elevated

Macrocytic anemia

ICD-9CM # 281.9

Anemia

Section III

Evaluate for etiology by Schilling’s test

Serum B12 normal folate decreased

Serum B12 decreased

Megaloblastic changes on peripheral blood smear (hypersegmented neutrophils)

ANEMIA, MACROCYTIC

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Diagnosis: thalassemia

Hemoglobin electrophoresis and globin chain synthesis studies abnormal

Diagnosis: sideroblastic anemia

Bone marrow aspirate has ringed sideroblasts

Diagnosis: anemia of chronic disease

Diagnosis: heavy metal poisoning (lead, mercury, cadmium)

Evaluate for infections, rheumatologic or malignant disease

Urine heavy metal screen negative

Diagnosis: autoimmune hemolytic anemia

Direct Coombs’ test positive

Diagnosis hereditary spherocytosis

Direct Coombs’ test negative

Spherocytes on peripheral blood smear

Urine heavy metal screen positive

Bone marrow aspirate does not have ringed sideroblasts

Evaluation for inherited red cell membrane defects

Abnormal red cell forms (nonspherocytes) on peripheral blood smear

Normal hemoglobin electrophoresis

Serum iron normal, ferritin normal, % serum iron saturation normal

Reticulocyte count elevated

FIGURE 3-7 Differential diagnosis of microcytic anemia. (From Rakel RE [ed]: Principles of family practice, ed 6, Philadelphia, 2002, WB Saunders.)

Evaluate for occult blood loss

Diagnosis: iron deficiency anemia

Serum iron decreased, ferritin decreased, % serum iron saturation decreased

Reticulocyte count not elevated

Microcytic anemia

ICD-9CM # 282.4

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ANEMIA, MICROCYTIC

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Anemia

Hereditary



Hb electrophoresis

Osmotic fragility

Abnormal Hb studies

Hb electrophoresis

Liver function

Other abnormalities

IAHA

Secondary (lymphoma, lupus, infections)

Coombs’ test

Enzyme analysis (G6PD, PK)



Spherocytes

Normal

Family history

R/O bleeding

Anemia with reticulocytosis

Test family for RBC morphology



Evaluate for AV shunts

Coagulation profile

Schistocytes

RBC morphology

Acquired



ICD-9CM # 790.99

PNH screen

LS-scan

Normal

Anemia

Section III CLINICAL ALGORITHMS

FIGURE 3-8 Evaluation of patients with hemolytic anemia. AV, Arteriovenous; Hb, hemoglobin; IAHA, idiopathic autoimmune hemolytic anemia; LS, liver spleen; PK, pyruvate kinase; PNH, paroxysmal nocturnal hemoglobinuria; RBC, red blood cell. (From Stein JH: Internal medicine, ed 5, St Louis, 1997, Mosby.)

Sickle cells

RBC morphology

Spherocytes

ANEMIA, WITH RETICULOCYTOSIS

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ANOREXIA ICD-9CM # 783.0 Patient with anorexia

Histor y Physical examination

Medical disease

Nonmedical etiology

Social factors

Medications

Consider: Sedatives and psychotropics Digoxin Laxatives Appetite suppressants Thiazide diuretics Levodopa Narcotics Antibiotics Miscellaneous

Diagnostic tests Chemistry panel CBC ESR Urinalysis Thyroid function tests Fasting plasma cortisol level ECG

CNS disease

Eating disorders

Psychologic disorders

Age-related conditions

Consider: Depression Dementia Alcoholism Drug abuse Psychosis Anxiety Bereavement

Consider: Decreased olfactor y sense Hypogeusia Visual disorders Hearing disorders Dental disorders

Endocrine disorders

GI disorders

Consider: Thyroid disease Adrenal insufficiency Diabetes Hyperparathyroidism Hypercalcemia

Consider: Malabsorption syndromes PUD Biliary disease Hepatitis Gastroesophageal reflux disease Esophageal motility disease Oral cavity problems GI malignancy

Other medical diseases

Consider: Chronic infection Malignancy Cardiopulmonar y disease Cerebrovascular disease Abdominal ischemia

Diagnostic tests: Stool for blood, fat, parasites Flexible sigmoidoscopy Upper GI with small bowel follow-through FIGURE 3-9 Evaluation of anorexia. CBC, Complete blood count; CNS, central nervous system; ECG, electrocardiogram; ESR, erythrocyte sedimentation rate; GI, gastrointestinal; PUD, peptic ulcer disease. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

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ARTHRALGIA LIMITED TO ONE OR FEW JOINTS ICD-9CM # 719.40 719.41 719.42 719.43 719.44 719.45 719.46 719.47

Arthralgia site NOS Arthralgia, shoulder region Arthralgia, upper arm Arthralgia, forearm Arthralgia, hand Arthralgia, pelvic region and thigh Arthralgia, lower leg Arthralgia, ankle and/or foot

Arthralgia limited to one or few joints

Complete history and physical examination

Significant trauma or focal bone pain

No

Yes Abnormal

Normal X-ray

Effusion or signs of inflammation? Yes

Probable inflammator y process

No

Joint aspiration

Fracture, tumor, or metabolic bone disease

Point tenderness or trigger points

Unsuccessful No

Successful

Yes

Bursitis, tendinitis, or fibromyalgia

>2000 WBCs? >75% PMNs? Yes

Coagulopathy, pseudogout, tumor, trauma, or Charcot’s joint

Osteoarthritis, internal derangement, soft tissue injury, or viral infection

No

Intraarticular fracture Crystals identified

Positive culture*

Sterile inflammatory joint fluid

Monosodium urate (gout); calcium pyrophosphate dihydrate (pseudogout)

Infectious arthritis

Suspect RA, viral arthritis, SLE, Lyme arthritis, sarcoidosis, or spondyloarthropathy

Check: PT/PTT, platelet count, bleeding time

*Synovial fluid culture as well as cervical, urethral, pharyngeal, and/or rectal evaluations for Neisseria gonorrhoeae and Chlamydia trachomatis when suspected.

Check: CBC, ESR, RF Consider: LFTs, HLA-B27, ANA, Lyme serologies, and pelvis radiographs

FIGURE 3-10 A diagnostic approach to arthralgia in a few joints. ANA, Antinuclear antibodies; CBC, complete blood count; ESR, erythrocyte sedimentation rate; LFTs, liver function tests; PMNs, polymorphonuclear neutrophils; PT, prothrombin time; PTT, partial thromboplastin time; RA, rheumatoid arthritis; RF, rheumatoid factor; SLE, systemic lupus erythematosus; WBCs, white blood cells. (Modified from American College of Rheumatology Ad Hoc Committee on Clinical Guidelines: Arthritis Rheum 39:1, 1996.)

CLINICAL ALGORITHMS

Bone marrow elements present

Bloody

Section III

Reevaluate

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ASCITES ICD-9CM # 789.5 Ascites NOS 197.6 Ascites, cancerous (malignant) M8000/6 457.8 Chylous 014.0 Tuberculous Ascites present by physical examination and/or ultrasound of abdomen

Diagnostic paracentesis: 1. Process fluid for LDH, glucose, albumin, total protein cell count and differential 2. Obtain Gram stain, AFB stain, bacterial and fungal cultures, amylase, and triglycerides on selected cases (suggested by history and physical examination) 3. If malignant ascites is suspected, consider CEA level and cytologic evaluation of paracentesis fluid 4. In suspected bacterial peritonitis, culture paracentesis fluid in blood culture bottles 5. Draw serum LDH, protein, albumin

Serum-ascites albumin gradient

Bloody fluid

Elevated amylase level

Elevated neutrophil count

Consider neoplasm or traumatic paracentesis

Pancreatic ascites

Consider infectious process

CT scan of abdomen, CEA, cytologic evaluation

CT scan of abdomen, ? ERCP

Obtain Gram stain, AFB stain, cultures, and start empiric antibiotic therapy

High gradient (≥1.1 g/dl)

Low gradient (<1.1 g/dl)

Cirrhosis, alcoholic hepatitis, cardiac failure, portal vein thrombosis, myxedema, Budd-Chiari syndrome

Pancreatic ascites, biliary ascites, nephrotic syndrome, peritoneal carcinomatosis, peritoneal tuberculosis, bowel obstruction/infarction

FIGURE 3-11 Evaluation of ascites. AFB, Acid-fast bacillus; CEA, carcinoembryonic antigen; CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; LDH, lactate dehydrogenase.

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ASTHMA, HOME MANAGEMENT ICD-9CM # 493.9 Asthma, unspecified 493.1 Intrinsic asthma 493.0 Extrinsic asthma Assess Severity Measure PEF: Value 50% personal best or predicted suggests severe exacerbation Note signs and symptoms: Degrees of cough, breathlessness, wheeze, and chest tightness correlate imperfectly with severity of exacerbation. Accessory muscle use and suprasternal retractions suggest severe exacerbation

Initial Treatment • Inhaled short-acting -agonist: up to three treatments of 2-4 puffs by MDI at 20-minute intervals or single nebulizer treatment

Good Response

Incomplete Response

Poor Response

Mild exacerbation PEF 80% predicted or personal best

Moderate exacerbation PEF 50%-80% predicted or personal best

Severe exacerbation PEF 50% predicted or personal best

No wheezing or shortness of breath

Persistent wheezing or shortness of breath

• Add oral corticosteroid • Add oral corticosteroid • Continue 2-agonist

• For patients on inhaled corticosteroids, double dose for 7-10 days

• Contact clinician urgently (this day) for instructions

• Contact clinician for follow-up instructions

• Repeat 2-agonist immediately • If distress is severe and nonresponsive, call your physician and proceed to emergency department; consider calling ambulance or 911

• Proceed to emergency department

FIGURE 3-12 Home management of acute asthma. MDI, Metered-dose inhaler; PEF, peak expiratory flow rate. (Modified from National Asthma Education and Prevention Program, National Heart, Lung, and Blood Institute, Expert Panel Report 2: Guidelines for the diagnosis and management of asthma, NIH Pub No 97-4051, July 1997.)

CLINICAL ALGORITHMS

• May continue 2-agonist every 3-4 hr for 24-48 hr

Section III

Response to 2-agonist sustained for 4 hr

Marked wheezing and shortness of breath

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BACK PAIN ICD-9CM # 847.9 Initial evaluation, especially vital signs, clinical findings

Stable “Red flags” in history should expand differential (see Table 3-1)

Unstable

Cardiac monitor Intravenous access Oxygen therapy Fluid bolus (20 ml/kg) Antibiotics if indicated ECG Chest x-ray

Unmonitored bed Analgesia Indication for imaging

Differential diagnosis is based on the initial history and physical examination

Differential diagnosis is based on the initial history and physical examination Acute back pain with neurologic deficits Obtain MRI Provide analgesia Rule out cauda equina Admit if unable to walk or perform ADLs Neurosurgical follow-up Bed rest for 2-4 days

Aortic dissection β-Blockade followed by IV antihypertensive Immediate surgical consult Transfer if needed Diagnostic tests Angiography Transesophageal Echocardiogram Spiral CT

Ruptured/expanding AAA Fluid resuscitation Immediately to the operating room if unstable Diagnostic tests if stable but symptomatic Bedside U/S Spiral CT

Cauda equina syndrome Emergent MRI Immediate neurosurgical consultation/transfer If due to a carcinoma, 100 mg dexamethasone Emergency oncology consultation for radiation or chemotherapy

Acute back pain without neurologic deficits Obtain plain X-rays if indicated Analgesia: NSAIDs preferred over opiates Limited bed rest for 1-2 days Exercise program

Epidural abscess or vertebral osteomyelitis Obtain CBC/ESR Obtain MRI (both) Bone scan (osteomyelitis) Immediate orthopedic and neurosurgical consultation Broad-spectrum antistaphylococcal antibiotics

FIGURE 3-13 Management of acute low back pain. AAA, Abdominal aortic aneurysm; ADL, activities of daily living; CBC, complete blood count; CT, computed tomography; ECG, electrocardiogram; ESR, erythrocyte sedimentation rate; IV, intravenous; NSAIDs, nonsteroidal antiinflammatory drugs. (From Marx JA [ed]: Rosen’s emergency medicine, ed 5, St Louis 2002, Mosby.)

TABLE 3-1

Red Flags for Potentially Serious Conditions

Possible Fracture From Medical History Major trauma, such as vehicle accident or fall from height Minor trauma or even strenuous lifting (in older or potentially osteoporotic patient)

Possible Tumor or Infection

Possible Cauda Equina Syndrome

Age over 50 or under 20 yr History of cancer Constitutional symptoms, such as recent fever or chills or unexplained weight loss Risk factors for spinal infection: recent bacterial infection (e.g., urinary tract infection); intravenous drug abuse; or immune suppression (from steroids, transplant, or human immunodeficiency virus) Pain that worsens when supine; severe nighttime pain

Saddle anesthesia Recent onset of bladder dysfunction, such as urinary retention, increased frequency, or overflow incontinence Severe or progressive neurologic deficit in the lower extremity

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BLEEDING, GASTROINTESTINAL ICD-9CM # 578.9 Patient encountered

Vital signs (BP, P); “stat” CBC, platelet count, prothrombin time, blood for type and crossmatch, intravenous catheters started, nasogastric lavage

Shock or postural vital sign changes

Admit ICU, urgent surgical and GI consultations, begin transfusions, resuscitate

No shock or vital sign changes

Admit regular hospital bed, evaluate for transfusion, elective GI consultation

Massive upper GI hemorrhage

Massive lower GI hemorrhage

Submassive upper GI hemorrhage

Submassive lower GI hemorrhage

Emergency endoscopy (6-12 hours)

Proctoscopy, emergency visceral angiogram, begin NG/oral PEG lavage (4 L) of colon

Endoscopy (within 24 hours of admission), begin empiric IV histamine-2 blockers

Begin NG/oral PEG colonic lavage (4 L), elective colonoscopy. If intermittent bleeding—technetium RBC scan

Sclerotherapy or band ligation, begin intravenous vasopressin/nitroglycerin or octreotide (25-50 µg/hr), standby Minnesota tube, Doppler ultrasound of hepatic/portal/splenic veins (evaluate for transjugular intrahepatic portosystemic shunt)

Endoscopic coagulation or injection of vessel, begin IV histamine-2 blockers

Continued bleeding

Stabilized/stopped bleeding

Surgery

Elective colonoscopy when prepared

FIGURE 3-14 Approach to the patient with gastrointestinal hemorrhage. BP, Blood pressure; CBC, complete blood count; GI, gastrointestinal; ICU, intensive care unit; IV, intravenous; NG, nasogastric; P, weight; PEG, percutaneous endoscopic gastrostomy; RBC, red blood cell. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

CLINICAL ALGORITHMS

Nonvariceal hemorrhage

Section III

Variceal hemorrhage

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BRADYCARDIA ICD-9CM # 427.89 Unspecified bradycardia 427.81 Chronic bradycardia 770.8 Newborn bradycardia 427.89 Postoperative bradycardia 337 Reflex bradycardia 427.89 Sinus bradycardia General approach to the patient with bradycardia

Asymptomatic

Symptomatic

Physiologic

Sinus node dysfunction*

No Rx

Clinical follow-up

Establish rhythm Dx AECG Event monitor Exercise test

Sinus node dysfunction*

AV block

1°

2°

No Rx

AV block

1°

High grade or complete

Rhythm—Sx correlation

Pacemaker

Pacemaker

Without BBB If type II consider EPS to evaluate site of block and/or pacemaker

No or unclear

Yes

Consider other cause Consider EPS† Consider pacemaker

Pacemaker

With BBB Consider EPS to localize site of block Consider pacemaker

2°, advanced or complete

Clinical follow-up

*Includes bradycardia-tachycardia syndrome. †EPS includes sinus node function and ventricular arrhythmia induction studies.

FIGURE 3-15 General approach to the patient with bradycardia. AECG, Ambulatory electrocardiography; AV, atrioventricular; BBB, bundle branch block; Dx, diagnostic; EPS, electrophysiologic study; Rx, treatment; Sx, symptoms; 1°, first-degree; 2°, second-degree. (From Goldman L, Braunwald E [eds]: Primary cardiology, Philadelphia, 1998, WB Saunders.)

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BREAST, RADIOLOGIC EVALUATION ICD-9CM # V76.10 1 History and physical examination

2 Bloody discharge?

Yes

2 Mammogram if none in past 6 months and age >35 years

2 Refer to surgeon

No Yes

2 Watery and unilateral? No 3 Prolactin and TSH assays

3 Mammogram if due for screening

Yes

3 Complete all radiologic recommendations

3 Refer to surgeon

Section III

3 Abnormal mammogram? No

Yes

3 Endocrine evaluation

No 4 Follow-up in 6 months

4 New abnormality?

Yes

4 Refer to surgeon

No 4 Inform patient of next screening date FIGURE 3-16 Breast cancer screening and evaluation. (From Institute for Clinical Systems Integration, Minneapolis: Postgrad Med 100:182-187, 1996.) Continued on following page

CLINICAL ALGORITHMS

3 Abnormal endocrine results?

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BREAST, RADIOLOGIC EVALUATION—cont’d FIGURE 3-16 (Continued) 1. Screening mammogram.* Patients are most commonly referred to a radiologist for screening mammography. Occasionally, however, patients are referred for diagnostic mammography based on symptoms or findings on breast exam. In the event of an abnormal finding on the mammogram, complete evaluation under the direction of a radiologist is recommended. It is the responsibility of the radiologist to complete the radiologic assessment so that the best possible characterization of the abnormality can be provided in an expeditious fashion to the primary care physician who ordered the original study. Any recommendations for referral to a surgeon for possible biopsy should be made directly to the primary care physician. The ultimate responsibility to make the referral will rest with the primary care physician. 2. Abnormal mammogram. Sorting abnormalities. Suspicious for cancer? On obtaining an abnormal finding on a mammogram, the radiologist determines whether further mammographic images are required for completion of the evaluation process. This may include a repeated image of the involved breast at 6 months to document stability of a low-risk, probably benign lesion. Alternatively, spot compression, magnification, or both may be necessary to obtain further characterization of indeterminate breast lesions. These additional studies should be done with the radiologist present to reduce the risk of patient recall for further studies necessary to evaluate the same lesion. On completion of these views, each and every abnormality uncovered for each independent lesion of the breast studied should be sorted according to the nature of the abnormality. The radiologist should classify the lesion as representing either suspicious microcalcifications, architectural distortion, or a soft-tissue mass. For any lesions identified as demonstrating microcalcifications that suggest cancer, biopsy will be recommended. It is up to the primary care physician to make the referral to a surgeon for biopsy. If a soft-tissue mass is identified on the mammogram, it should be studied further to determine its relative risk for malignancy. Any suspect lesions identified as having associated microcalcifications, architectural distortion, or interval growth when compared with the previous mammogram should likewise be referred to a surgeon for possible biopsy. 3. Ultrasound results. When the mass is not immediately suggestive of cancer, an ultrasound should be performed to determine whether the lesion is solid. A solid mass should be further characterized for its level of benignity according to three criteria: • Size less than 15 mm • Three or fewer lobulations • More than 50% of the margin of the lesion appearing well circumscribed in any view Patients who have lesions that fit all three criteria may be observed and then evaluated with a 6-month follow-up study. Any lesion that does not fit all three criteria for benignity should be characterized as indeterminate, and biopsy should be considered. Likewise, any solid mass that is palpable should be referred to a surgeon for possible open biopsy. Finally, any lesion that appears to be new since the last screening mammogram should be considered for biopsy. 4. Aspiration and results. If the ultrasound of the soft-tissue mass demonstrates that it is a cystic lesion, the cyst should be further categorized by the criteria listed in the algorithm: irregular wall, as seen on ultrasonography; internal echoes; complex, septated appearance; and palpability within the region of the ultrasound-proven cyst. A positive finding for any of these criteria would be an indication for ultrasound-directed aspiration of the cyst. Aspiration should also be offered if the patient requests it. After cyst aspiration, a single-view mammogram should be obtained to demonstrate complete resolution of the lesion. If the lesion is sufficiently complex, a cyst pneumogram may be performed. Should any residual mass be present or if the cyst pneumogram findings are abnormal, biopsy should be recommended. If, on the other hand, the mass is a simple cyst that does not fit any of the previously listed criteria, the patient should be returned to the screening process, and completion of this evaluation should be reported to the ordering health care provider.

*ICSI healthcare guidelines are designed to assist clinicians by providing an analytic framework for the evaluation and treatment of patients. They are not intended either to replace a clinician’s judgment or to establish a protocol for all patients with a particular condition. A guideline will rarely establish the only approach to a problem. In addition, guidelines are “living documents” that are expected to be imperfect and are subject to annual review and revision. ICSI is a nonprofit organization that provides healthcare quality improvement services to 20 medical groups affiliated with HealthPartners in central and southern Minnesota and western Wisconsin. The guidelines are developed through a process that involves physicians, nurses, and other healthcare professionals from beginning to end, and healthcare purchasers are included in decision making. To order any of the more than 40 guidelines ICSI has developed, contact the ICSI Publications Fulfillment Center, in care of the ARDEL Group, 6518 Walker St., Suite 150, Minneapolis, MN 55426; 612-927-6707.

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BREAST, ROUTINE SCREEN OR PALPABLE MASS EVALUATION

295 ICD-9CM # 611.72 Breast mass or lump, nonpuerperal

1 History and physical examination

2 Palpable mass?

Yes

No 6 Is screening mammogram due?

2 Dominant mass?

No

Yes

3 Aspirate mass or refer to appropriate consultant fo raspiration

4 Residual mass or bloody tap?

Yes

4 Refer to surgeon

No 6 Is screening mammogram due?

No

4 Mammogram if none in last 6 months and age >35 years

Yes

No

Yes

6 Mammogram

5 Inform patient of next screening date

5 Breast imaging

No No

6 Inform patient of next screening date

5 Abnormal breast imaging?

No

Yes Yes

FIGURE 3-17 Breast cancer screening and evaluation. (From Institute for Clinical Systems Integration, Minneapolis: Postgrad Med 100:182, 1996.) 1. History and physical examination.* Primary care evaluation is initiated with history taking aimed at uncovering and characterizing any breast-related symptom. A risk assessment should also be undertaken for identified risk factors, including patient age over 50 years, any past personal history of breast cancer, history of hyperplasia on previous breast biopsies, and family history of breast cancer in first-degree relatives (mother, sister, daughter). Physical examination should include inspection of the breast for any evidence of ulceration or contour changes and inspection of the nipple for Paget’s disease. Palpation should be performed with the patient in both the upright and supine positions to determine the presence of any palpable mass. 2. Palpable mass? Dominant mass? A dominant mass is a palpable finding that is discrete and clearly different from the surrounding parenchyma. If a palpable mass is identified, it should be determined whether it represents a dominant (i.e., discrete) mass, which requires immediate evaluation. The primary care physician or appropriate consultant should attempt to aspirate any dominant mass because a simple cyst may be uncovered, in which case aspiration completes the evaluation process. 3. Aspirate mass or refer for aspiration. Aspiration of a dominant palpable mass should be performed by the primary care physician or by the appropriate consultant. The breast skin is prepped with alcohol. Then, with the lesion immobilized by the nonoperating hand, an 18- to 25-gauge needle mounted on a 10-ml syringe is directed to the central portion of the mass for a single attempt at aspiration. Successful aspiration of a simple cyst would yield a nonbloody fluid with complete resolution of the dominant mass. Typical watery fluid may be discarded. However, cyst fluid that is bloody or unusually tenacious should be examined cytologically. Continued on following page

Yes 5 Refer to surgeon

CLINICAL ALGORITHMS

6 Complete all radiologic recommendations

5 Residual mass?

Section III

6 Abnormal mammogram?

5 Follow-up clinical breast examination in 1 month

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BREAST, ROUTINE SCREEN OR PALPABLE MASS EVALUATION—cont’d FIGURE 3-17 (Continued) 4. Residual mass or bloody tap? Mammogram if none in past 6 months. Refer to surgeon. Should the mass remain after the attempt at aspiration or should frank blood be aspirated during the process, the presence of a malignant process cannot be ruled out. Patients with a residual mass or bloody tap should be referred to a surgeon for possible biopsy. Before the referral, a mammogram should be obtained for any patient over age 35 years who has not had a mammogram within the preceding 6 months. In patients 35 years and under, obtaining any other breast-imaging studies should be left to the discretion of the surgeon or radiologist. 5. Is screening mammogram due? Breast imaging. Follow-up clinical breast examination. Refer to surgeon. Should physical examination demonstrate a palpable mass that is not clearly a discrete and dominant mass, its size, location, and character should be documented in anticipation of a followup examination. A screening mammogram should be obtained if one has not been done within the recommended interval. If no mammogram is required or if a required mammogram demonstrates no abnormality, a follow-up examination in 1 month is indicated. Should any residual mass be identified, the patient should be referred to a surgeon for possible biopsy. Patients with a persisting nondominant palpable mass that does not resolve within 1 month and those with any recurring cystic mass should be referred for surgical evaluation. If no mass is apparent at the time of the follow-up examination, the patient should then be informed of the appropriate date for her next screening examination, according to the recommended intervals. 6. Screening mammogram and results. After completion of the physical examination, the appropriateness of a routine screening mammogram should be determined. If a mammogram is done, the radiologist should provide the results to the primary care physician for reporting to the patient. Should any abnormalities be uncovered, it will be the responsibility of the radiologist to complete any additional imaging studies required for the complete radiographic characterization of the lesion. The radiologist should make certain that all recommended additional views, follow-up studies, and ultrasound examinations have been completed before referral to a surgeon. However, it is important that the primary care physician who ordered the mammogram review the results of these studies to understand fully the opinion of the radiologist and to ensure that all recommendations of the radiologist have been completed. Should the radiologist recommend that surgical consultation is warranted, it will be the responsibility of the primary care physician to establish this referral. NOTE: The importance of communication between the surgical consultant and the primary care physician cannot be overstated. Biopsy results should be reported both to the surgeon and to the primary care physician. More important, patients who do not require biopsy after surgical consultation should be returned to the routine screening process. This process is under the supervision of the primary care physician. Therefore it is absolutely necessary for the primary care physician to know when the patient reenters the routine screening population. In the event that new symptoms arise during the screening interval, the patient should be evaluated by the primary care physician using the primary care evaluation process of this guideline.

*ICSI healthcare guidelines are designed to assist clinicians by providing an analytic framework for the evaluation and treatment of patients. They are not intended either to replace a clinician’s judgment or to establish a protocol for all patients with a particular condition. A guideline will rarely establish the only approach to a problem. In addition, guidelines are “living documents” that are expected to be imperfect and are subject to annual review and revision. ICSI is a nonprofit organization that provides healthcare quality improvement services to 20 medical groups affiliated with HealthPartners in central and southern Minnesota and western Wisconsin. The guidelines are developed through a process that involves physicians, nurses, and other healthcare professionals from beginning to end, and healthcare purchasers are included in decision making. To order any of the more than 40 guidelines ICSI has developed, contact the ICSI Publications Fulfillment Center, in care of the ARDEL Group, 6518 Walker St., Suite 150, Minneapolis, MN 55426; 612-927-6707.

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CARDIOMEGALY ON CHEST X-RAY ICD-9CM # 429.3 Idiopathic cardiomegaly 746.89 Congenital cardiomegaly 402.0 Hypertensive cardiomegaly, malignant 402.1 Hypertensive cardiomegaly, benign 402.11 Hypertensive cardiomegaly with congestive heart failure Evaluation of cardiomegaly on chest x-ray

Review history Examination ECG

Echocardiogram

Left ventricular dilation

Biventricular dilation

Right ventricular dilation

Evaluate for: Valvular heart disease Coronary artery disease Cardiomyopathy

Consider right heart failure secondary to left heart failure from various causes of left ventricular dilation

Pulmonary hypertension?

? Pericardiocentesis

? Cause

No further workup

Consider: CT MRI Surgery/biopsy

No Evaluate for: Atrial septal defect Tricuspid regurgitation

FIGURE 3-18 Approach to the patient with cardiomegaly. When cardiomegaly is found on the chest radiograph, the history and physical examination should be reviewed and an electrocardiogram (ECG) performed before obtaining a two-dimensional Doppler echocardiographic study. Cardiomegaly may be explained by left ventricular dilation, biventricular dilation, right ventricular dilation, or pericardial abnormalities, or it may be found to be spurious on the echocardiogram. Rarely, isolated abnormalities of the atrium, particularly the left atrium, may cause abnormalities on the chest radiograph but will not cause true cardiomegaly. Depending on the echocardiographic findings, further tests can help elucidate the cause of echocardiographically confirmed cardiomegaly. CT, Computer tomography; MRI, magnetic resonance imaging; R/O, rule out. (From Goldman L, Branwald E [eds]: Primary cardiology, Philadelphia, 1998, WB Saunders.)

CLINICAL ALGORITHMS

Evaluate for: Pulmonary emboli Mitral stenosis Primary pulmonary hypertension Eisenmenger‘s syndome

R/O tamponade

No abnormalities

Section III

Yes

Pericardial effusion/thickening/mass

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CEREBRAL ISCHEMIA ICD-9CM # 437.1 Cerebral ischemia (chronic) 435.9 Cerebral ischemia intermittent (transient) Patient with cerebral ischemia

History, examination ? Nonlacunar involvement of cortex or cerebellum ? Evidence of multiple cerebral artery territories affected ? Evidence of systemic embolism ? Syncope or palpitations at onset or irregular pulse ? Rapid resolution of neurologic deficit ? Young patient without premature atherosclerosis ? History of cardiac disease

Anterior circulation cerebral ischemia and ipsilateral >70% carotid stenosis

Surgical evaluation for carotid endarterectomy

Suspected cardioembolic source of embolism

Definite cardioembolic source of embolism

Neuroimaging–CT or MRI ECG, CXR

Neuroimaging–CT or MRI ECG, CXR

Fever, systemic symptoms

Evaluation for bacterial endocarditis

Cardioembolic source?

Yes

TIA Small cerebral infarct Mild or moderate neurologic deficit

No

Initiate heparin anticoagulation

Transthoracic echocardiogram

Cardioembolic source?

Moderate or large cerebral infarct Moderate to severe neurologic deficit Evidence of hemorrhagic transformation of bland infarct

Yes

No

Defer anticoagulation and clinically reevaluate daily, weighing the risks of reembolization vs. hemorrhagic transformation

Transesophageal echocardiogram

Cardioembolic source?

Yes

No Additional rhythm monitoring (telemetry, Holter monitoring) FIGURE 3-19 Evaluation of patients with cerebral ischemia for a cardioembolic source. CT, computed tomography; CXR, chest radiograph; ECG, electrocardiogram; MRI, magnetic resonance imaging; TIA, transient ischemic attack. (From Johnson R [ed]: Current therapy in neurologic disease, ed 5, St Louis, 1997, Mosby.)

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CHRONIC OBSTRUCTIVE PULMONARY DISEASE ICD-9CM # 496 COPD 492.8 Emphysema

Ipratropium bromide MDI 3-6 puffs qid Suboptimal clinical response Add: 2-agonist MDI 2 puffs qid  prn Not to exceed 24 puffs/day Suboptimal clinical response Consider methylxanthines such as aminophylline and theophylline only in patients who do not respond to other bronchodilators Suboptimal clinical response Trial: oral prednisone 40 mg once daily (average adult) Spirometry before initiating prednisone

Metered-dose inhaler (MDI)

Educate patient as to proper technique

Assess need for O2 at rest; exercise and sleep if clinically indicated

Cough  tenacious sputum?

Consider trial of expectorants or mucolytics ? Inhaled DNase

Acute infectious exacerbations of bronchitis 4 per year?

Consider prophylactic antibiotic therapy

Strong features of asthmatic bronchitis?

Early trial of inhaled and oral corticosteroid before theophylline

Active smoker?

Nicotine replacement therapy or Zyban plus smoking cessation program

Poor exercise tolerance?

Pulmonary rehabilitation

Secondary erythrocytosis?

Check need for supplemental oxygen; phlebotomy to Hct 52%

Severe emphysema  bullous abnormalities or hyperinflation?

Consider surgery: bullectomy, lung reduction, or transplantation

FIGURE 3-20 Managed care guide: pharmacotherapy and general management approaches for chronic obstructive pulmonary disease (COPD). DNase, Deoxyribonuclease; Hct, hematocrit; prn, as needed; qid, four times a day; qod, every other day. (Modified from Noble J: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

CLINICAL ALGORITHMS

Use qod dosing and add inhaled corticosteroid in attempt to decrease prednisone

Rapidly taper prednisone

“Breath-activated” aerosol inhaler (pirbuterol)

Section III

Taper oral dose to minimal possible

No significant improvement

Inability to coordinate conventional MDI/spacer

Hypoxic?

Repeat spirometry in 2 weeks Significant improvement

Use spacer device to enhance drug deposition in lungs

Constipation

Endoscopy normal

Perform proctosigmoidoscopy

Endoscopy abnormal

Extrinsic compression

Perform barium enema

Metabolic tests abnormal

BE abnormal

Barium studies normal

Metabolic tests normal

Measure TSH, Ca, K

Cathartic colon Megacolon Neoplasm Stricture Volvulus

Diverticulitis Melanosis coli Anal stenosis Fecal impaction

Diuretics Ferrous sulfate Narcotics Anticholinergics Calcium-containing Antacids Aluminum-containing Antidepressants Anticonvulsants Laxative/enema abuse Rectal carcinoma Barium sulfate Fissures Phenothiazines Stricture Antihypertensives Abscess Lead poisoning Hemorrhoids Abdominal/pelvic tumor Perineal abscess

Hyperparathyroidism Milk-alkali syndrome

Depression Victim of sexual abuse Multiple sclerosis Muscular dystrophy Spinal cord injury/tumor CNS tumors Parkinson’s disease Dementia Cerebral palsy CVA Scleroderma Dermatomyositis

Hypothyroidism Hypercalcemia Hypokalemia

Irritable bowel syndrome Idiopathic constipation Poor bowel habits Chronic laxative use Porphyria

Metabolic disorder

Acquired Idiopathic Hirschsprung’s disease Chagas’ disease

Hypothyroidism

Collagen-vascular disease

Diabetes mellitus

Neurologic disorders

Psychiatric disorders

FIGURE 3-21 Constipation. BE, Barium enema; CNS, central nervous system; CVA, cerebral vascular accident; TSH, thyroid-stimulating hormone. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

History/exam unrevealing

Perform history and physical exam

History/exam abnormal

Hermorrhoidal disease Poor diet/fluid intake Debilitation/inactivity Intercurrent disease Fecal impaction Previous abdominal/ perineal surgery Drugs Rectal/colon carcinoma

ICD-9CM # 564.0

300

CONSTIPATION

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Constipation

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COUGH, CHRONIC ICD-9CM # 786.2 Cough  3 weeks

History/physical examination

Diagnosis not obvious

Chest x-ray

Spirometry

Recent URI, otherwise normal Reassure

PND signs/symptoms Therapeutic trial

Normal

Methacholine challenge

Further GERD evaluation –pH probe or therapeutic trial

Further cardiac evaluation ? ECG ? Echocardiogram ? Stress test ? Cardiac catheterization

Further pulmonary evaluation ? Computed tomography of chest ? Bronchoscopy

FIGURE 3-22 Diagnostic approach to chronic cough. ECG, Electrocardiogram; GERD, gastroesophageal reflux disease; PND, paroxysmal nocturnal dyspnea; UGI, upper gastrointestinal tract; URI, upper respiratory infection. (From Carlson KJ et al: Primary care of women, ed 2, St Louis, 2002, Mosby.)

CLINICAL ALGORITHMS

Further GI evaluation ? Endoscopy ? UGI series ? Barium swallow

Section III

Diagnosis still uncertain Consider referral to specialist

Cyanosis

Check physical exam

Decreased oxygen saturation

Normal oxygen saturation

Check phonation

Acrocyanosis

Normal phonation

Difficulty with phonation

Check cardiac exam

Check CXR

No characteristic murmurs

Abnormal CXR

CXR normal

Cyanotic heart disease Characteristic murmurs

Upper airway obstruction

V/Q scan normal Abnormal hemoglobin

Pulmonary A-V malformations

Emphysema Bronchitis Hyperinflation Mass lesions

Pneumonia Pulmonary edema Interstitial fibrosis Interstitial pattern

Congestive heart failure

Check V/Q scan

V/Q scan abnormal

Pulmonary embolus

FIGURE 3-23 Cyanosis. A-V, Arteriovenous; CXR, chest x-ray; V/Q, ventilation-perfusion. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

Check oxygen saturation (CO-oximetry)

Generalized cyanosis

Localized cyanosis

Arterial occlusion Congenital heart disease

Localized Upper extremity—transposition of great arteries Lower extremity—patent ductus arteriosus

Raynaud’s phenomenon/disease Congestive heart failure Vasoconstricting drugs

Peripheral

ICD-9CM # 782.5 Cyanosis NOS

302

CYANOSIS

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Cyanosis

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DELIRIUM, GERIATRIC PATIENT ICD-9CM # 293.0 Delirium, acute 292.81 Delirium, drug induced 293.1 Delirium, subacute 293.81 Delirium, transient organic with delusions 293.82 Delirium, transient organic with hallucinations

Establish mental status diagnosis

Suspected mental status change

Establish patient’s baseline cognitive functioning and clinical course of change

Chronic

Acute

Dementia evaluation

Cognitive assessment (including delirium rating)

• Depression • Acute psychotic disorder • Other psychiatric disorder

• History, including alcohol use • Vital signs • Physical examination, including neurologic examination • Targeted laboratory testing (creatine, glucose, sodium, ALT, calcium, CBC) • Search for occult infection • Oxygen saturation

Remove or alter any potentially contributory medications, or any with potentially harmful interactions

Potential contributor identified

No potential contributor identified

Evaluation and treatment as appropriate for each contributor

Consider further options • Laboratory testing: TFTs, B12, toxicology screen, ammonia level • Cerebrospinal fluid examination • Brain imaging • Electroencephalogram

Delirium management

Manage delirium symptoms

All patients

Nonpharmacologic approaches • Reorientation strategies and schedule of activities • Use of sitters or family members • Relaxation techniques, music, massage • Avoid physical restraints and immobilizing devices (e.g., Foley catheters) • Maintain mobility and self-care • Use eyeglasses, hearing aids, interpreters • At night, keep room quiet with low-level lighting • Allow uninterrupted period of sleep at night

Evaluation and treatment as appropriate for each condition

Subgroup with severe agitation

Pharmacologic approaches Indications: reserved for patients with severe agitation that will: 1) Cause interruption of needed medical therapies (e.g., intubation) 2) Pose safety hazard to patient or staff Treatment: • Haloperidol 0.5-1.0 mg IM or resperidone 0.25-0.5 mg PO or quietapine 25-50 mg PO • Repeat dose q30min until sedation achieved (maximum haloperidol 3-5 mg/24 hr or resperidone 2 mg/24 hr or quietapine 600 mg/24 hr) • Maintenance: 50% loading dose in divided doses over next 24 hours • Taper dose over next few days

FIGURE 3-24 Algorithm for evaluation of suspected mental status change in an older patient. ALT, Alanine aminotransferase; CBC, complete blood count; IM, intramuscular; NG, nasogastric; PO, by mouth; PRNs, as needed; TFTs, thyroid function tests. (Adapted from Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

CLINICAL ALGORITHMS

Review medication list (including PRNs and over-the-counter medications)

Section III

Identify and treat underlying causes

Delirium

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DEMENTIA ICD-9CM # 290.10 Dementia, presenile 290.0 Dementia, senile 437.0 Dementia, arteriosclerotic Patient with dementia

Screening tests (Table 3-2)

Reversible dementia

Irreversible dementia

Multiinfarct dementia

Specific treatment available

Hydrocephalus

Cardiac source of emboli?

Yes

No

Communicating

Warfarin

Aspirin, clopidogrel, warfarin, or carotid endarterectomy

VP/VA shunt

FIGURE 3-25

TABLE 3-2

CT scan or MRI

Noncommunicating

Supportive/ symptomatic treatment

Management of dementia. VA, ventriculoatrial; VP, ventriculoperitoneal.

Screening Tests for Diagnosis of Dementia

Test

Rationale

Blood Test Complete blood count Creatine, ALT Serum B12 level

Assess general nutritional status Exclude renal or hystic disease Exclude vitamin B12 deficiency

TSH  free T4 or TSH  FTI HIV serology Calcium Cerebrospinal Fluid Cell count/protein level Cytology VDRL

Exclude primary and secondary hypothyroidism Exclude HIV infection Exclude hypercalcemia or hypocalcemia Exclude chronic meningitis Exclude carcinomatous meningitis Exclude neurosyphilis

Remarks

Consider antiparietal cell antibody or Schilling’s test if B12 level is low Perform only if indicated; consent from patient required

Perform only if indicated Perform only if indicated Perform only if indicated; check serum TPHA and HIV serology if CSF VDRL is positive

CT Scan/MRI of the Brain Identify infarcts and white matter changes; exclude presence of neoplasm, demyelinating disease, and hydrocephalus; location of atrophy may suggest the diagnosis (e.g., parahippocampal atrophy in Alzheimer’s disease, frontotemporal atrophy in Pick’s disease) Electroencephalogram Exclude metabolic encephalopathies; useful if CreutzfeldtJakob disease or status epilepticus is suspected Neuropsychologic Evaluation Help to characterize pattern of cognitive impairment, which may aid in the classification of dementia; rule out pseudodementia from depression

Perform only if indicated

From Johnson RT, Griffin JW: Current therapy in neurologic disease, ed 5, St Louis, 1997, Mosby. ALT, Alanine aminotransferase; CSF, cerebrospinal fluid; CT, computed tomography; FTI, free thyroxine index; HIV, human immunodeficiency virus; MRI, magnetic resonance imaging; T4, thyroxine; TPHA, Treponema pallidum hemagglutination assay; TSH, thyroid-stimulating hormone; VDRL, Venereal Disease Research Laboratory test.

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DEPRESSION ICD-9CM # 296.2 Major depressive disorder, single episode Evaluate and make diagnosis

Select and initiate treatment with SSRI. Counsel and educate patient about the medication, side effects, duration of treatment, and compliance.

Suicidal ideation, psychosis, history of mania, recurrent or refractory depression, complex psychiatric diagnosis, substance abuse.

Monitor acute treatment (every 1-2 weeks)

Immediate referral to a psychiatrist

Assess response (week 6)

Clearly better

Somewhat better

Not better at all

Continue treatment for 6 more weeks

Continue treatment (adjust dosage)

Augment or change treatment

Monitor treatment (every 1-2 weeks)

Section III

Clearly better

Assess response (week 12)

Complete remission?

Yes

Medication continued for 4-9 months Consider maintenance treatment

No Relapse?

Refer or consult a psychiatrist or other mental health professional

Change treatment

FIGURE 3-26 Guidelines for the treatment of depression in the primary care setting. SSRI, Selective serotonin reuptake inhibitor. NOTE: Time of assessment (weeks 6 and 12) rests on very modest data. It may be necessary to revise the treatment plan earlier for patients who fail to respond. (From AHCPR Quick Reference Guide of Clinicians, No. 5: Depression in primary care: Detection, diagnosis and treatment, 1993; and American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, Washington, DC, 1994, American Psychiatric Association.)

CLINICAL ALGORITHMS

Not better

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DIARRHEA, ACUTE ICD-9CM # 787.91 1.

Physical examination

Hydrate as necessary

2.

Stool examination

Inflammatory cells

Ova and parasites

Blood: if present, consider:

Present: suggests mucosal disease a. IBD b. Invasive bacterial infections (such as Shigella spp., Salmonella spp., amebiasis, Campylobacter) Absent: suggests viral gastroenteritis, toxin (Staphylococcus, Escherichia coli, Aeromonas, or Plesiomonas spp.), or drug-related diarrhea or IBS

3.

a. IBD b. Bacterial infections: Salmonella spp. Shigella spp. Amebiasis Campylobacter Clostridium difficile toxin E. coli O157:H7

Culture stool

Positive culture result

Negative culture result

Treat appropriately, except for Salmonella infections in which treatment may prolong the carrier state

See step 5

4.

Flexible sigmoidoscopy

Abnormal mucosa

Normal mucosa

a. Pseudomembranes: check for C. difficile toxin: Wait for culture results treat with metronidazole, vancomycin, or bacitracin b. Ulcerations/granularity (1) Proctitis only: culture for Chlamydia trachomatis, Neisseria gonorrhoeae; Gram stain and culture urethra and pharynx; biopsy as in (2) (2) More extensive: culture; biopsy to look for amebae, granulomas, or nonspecific finding of IBD

5. Inflammatory cells in stool IBD likely

Negative stool culture result No inflammatory cells in stool

a. If history is appropriate, with travel to endemic areas, or if patient has a. Severely ill: rule out toxic megacolon; hypogammaglobulinemia, evaluate analyze blood cultures; abdominal x-ray; duodenal aspirate for Giardia treat as IBD b. Stop all drugs, stop milk products, rule b. Not severely ill: barium studies or out malabsorption, observe, and treat colonoscopy after careful and gentle symptomatically; if symptoms persist or preparation recur, perform barium studies or colonoscopy FIGURE 3-27 Diagnostic steps in the assessment of acute diarrhea. IBD, Inflammatory bowel disease; IBS, irritable bowel syndrome. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

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DIARRHEA, CHRONIC ICD-9CM # 787.91 1.

Diagnostic steps 1 to 4 as in Fig. 3-27

a. Results diagnostic for infectious diarrhea (uncommon in chronic diarrhea except for Clostridium difficile after antibiotics), inflammatory bowel disease, or overt druginduced diarrhea b. Results nondiagnostic; usually without inflammatory cells in stool 2.

Stool volume

a. Small volume: usually seen in infectious diarrhea or inflammatory bowel disease (consider colonoscopy), but can also be seen in malabsorption syndromes and irritable bowel syndrome b. Large volume: suggests malabsorption syndromes, secretory diarrhea, or laxative abuse 3.

Stool Sudan stain

Positive

Negative

Suggests malabsorption syndrome, pancreatic insufficiency, bile salt insufficiency, or mucosal disease

See step 4

4.

Oral intake stopped

a. Malabsorption syndromes: stool osmolality > plasma osmolality b. Laxative ingestion: see step 5 c. Congenital chloridorrhea (1) Stool electrolytes: chloride concentration greater than the sum of sodium and potassium concentrations in stool water (2) No fecal osmotic gap

5.

Laxative abuse detection

a. Screening tests (1) Detailed history (2) Sigmoidoscopy and biopsy for melanosis coli (3) Barium enema: dilated, hypomotile “cathartic colon” b. Specific tests (1) Urine screening test for senna (2) Chromatographic test for bisacodyl (3) Stool test for fecal sulfate and phosphate (4) Magnesium concentration in fecal water (atomic absorption spectrophotometry) 6.

Radiologic studies

Perform barium studies only after stool examination, culture, and studies requiring quantitative measurements of the stool have been completed. FIGURE 3-28 Diagnostic approach to the patient with chronic diarrhea. 5-HIAA, 5-Hydroxyindoleacetic acid; VIP, vasoactive intestinal polypeptide. (Modified from Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Diarrhea stops

Section III

Diarrhea continues a. Secretory diarrhea: stool osmolality = stool (Na+ + K+) x 2 b. Nasogastric suction (1) Diarrhea stops (a) Zollinger-Ellison syndrome: gastric analysis, gastrin, secretin stimulation (b) Laxative abuse: see step 5 (2) Diarrhea continues (a) Secretory diarrhea: plasma VIP, calcitonin, urinary 5-HIAA abdominal ultrasound, computed tomography, and/or selective mesenteric angiogram to identify tumor (b) Laxative abuse: see step 5

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DYSPEPSIA ICD-9CM # 563.3 Dyspepsia atonic 536.8 Dyspepsia disorders other unspecified function of stomach 306.4 Dyspepsia, psychogenic Dyspepsia

Clinical evaluation

45 years old and no alarming features

45 years old and alarming features (e.g., severe pain, dysphagia, weight loss, vomiting, bleeding)

Hp test (serology or breath test) Positive

Negative

Treat Hp

Empiric trial (e.g., antisecretory or prokinetic drug)

Symptoms resolve Fails

Exclude by history GERD IBS Aerophagia Manage appropriately

Fails

Endoscopy

Structural diseases including Hp

Functional dyspepsia Needs a drug trial?

Treat appropriately

Follow-up

Antisecretory or prokinetic drug

Evaluate in 4 weeks

Success • Stop drug

Failure

Switch to alternate therapy

Success • Stop drug

Failure Reevaluate clinically Additional options of uncertain efficacy • Behavioral/psychotherapy • Antidepressant

FIGURE 3-29 Algorithm for the evaluation of dyspepsia. GERD, Symptomatic gastroesophageal reflux disease; Hp, Helicobacter pylori; IBS, irritable bowel syndrome. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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DYSPHAGIA ICD-9CM # 787.2 Dysphagia

Food stops or "sticks" after swallowed

Difficulty initiating swallows (includes coughing, choking, and nasal regurgitation)

Esophageal dysphagia (requires barium swallow or endoscopy)

Oropharyngeal dysphagia (consider modified barium swallow)

Solid food only

Solid or liquid food

Mechanical obstruction

Neuromuscular disorder

Intermittent

Progressive

Age >50 Weight loss

Lower esophageal ring

Peptic stricture

Carcinoma

Chest pain

Chronic heartburn

Bland regurgitation Weight loss

Diffuse Scleroderma Achalasia esophageal spasm FIGURE 3-30 Differential diagnosis of dysphagia. (Adapted from Andreoli TE [ed]: Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders.)

CLINICAL ALGORITHMS

Chronic heartburn No weight loss

Progressive

Section III

Bread/steak

Intermittent

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DYSPNEA, ACUTE ICD-9CM # 786.00 What is the duration of dyspnea?

Acute (emergent)

Chronic (subacute)

Follow algorithm for acute dyspnea

Follow algorithm for chronic dyspnea (Fig. 3-32)

Acute dyspnea

Relevant history of present illness Relevant past medical histor y Focused physical examination

Diagnosis evident?

A

No

Yes: Examples: Chest trauma Choking Upper airway obstruction

Order appropriate diagnostic tests (from Level I, Fig. 3-31, B)

Specific treatment

Diagnosis evident?

Yes: Examples: Pneumothorax Acute pulmonary edema Acute respirator y distress syndrome Pneumonia Bronchial asthma Exacerbation of COPD Acute myocardial infarction Pleural effusion Hyperventilation syndrome Sepsis

Specific treatment

No

Order appropriate diagnostic tests (from Level II, Fig. 3-31, B)

Diagnosis evident?

Yes: Examples: Pulmonary thromboembolism Bronchial obstruction

No Order appropriate additional diagnostic tests (from Level II or III, Fig. 3-31, B) Consider consultation

Specific treatment FIGURE 3-31 A, Evaluation of the patient with dyspnea. COPD, Chronic obstructive pulmonary disease. (From Stein J [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

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DYSPNEA, ACUTE—cont’d Foundation Thorough medical history with emphasis on the respiratory system Complete physical examination

Supplemental tests if necessary Level I: Posteroanterior and lateral chest radiograph Pulmonary function tests Pulse oximetr y Arterial blood gases Electrocardiogram Sputum examination Clinical laboratory tests

B Level II: Fiberoptic bronchoscopy Thoracentesis Ventilation/perfusion lung scintigraphy Pulmonary exercise stress testing Computed tomography Magnetic resonance imaging

CLINICAL ALGORITHMS

FIGURE 3-31 (Continued) B, Medical history and physical examination are the foundation for the diagnosis of respiratory system disease. Diagnostic tests of increasing levels of complexity and invasiveness are performed if necessary to supplement the initial history and physical examination. (From Stein J [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

Section III

Level III: Invasive diagnostic procedures Right heart catheterization Pulmonary angiography Needle biopsy of lung or pleura Thoracic surger y

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DYSPNEA, CHRONIC ICD-9CM # 786.00 Chronic dyspnea

Complete medical histor y Thorough physical examination Order appropriate tests for diagnosis or confirmation (from Level I, Fig. 3-31, B)

Diagnosis evident?

Yes: Examples: Bronchial asthma Chronic obstructive pulmonary disease Pleural effusions or fibrosis Diffuse infiltrative lung disease Atelectasis Severe anemia Kyphoscoliosis Diaphragm paralysis

Specific treatment

No Order appropriate diagnostic tests (from Level II, Fig. 3-31, B)

Diagnosis evident?

Yes: Examples: Congestive heart failure Mitral stenosis Neuromuscular disease Pulmonary hypertension Lung cancer Chronic pulmonary thromboembolism Obesity Physical deconditioning

Specific treatment

FIGURE 3-32

No

Order appropriate additional diagnostic tests (from Level II or III, Fig. 3-31, B) Initiate clinical trial of specific therapy Consider pulmonary exercise stress test Consider psychogenic dyspnea Consider consultation

Chronic dyspnea. (From Stein J [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

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EDEMA, GENERALIZED ICD-9CM # 782.3 Edema NOS 782.3 Edema, lower extremities Edema History Physical examination Generalized edema

Regional edema (see Fig. 3-34)

Check JVP

JVP normal

JVP elevated

Check serum albumin

Chest radiography

Cardiomegaly

Normal heart size

Check echocardiogram

Evaluate lung fields

Pericardial effusion

Poor contractibility

Pericarditis/ tamponade

CHF

Normal TFTs

Proteinuria

Abnormal urine sediment

Check BUN/ creatinine Check TFTs

Decreased serum albumin

Evaluate for renal pathology

Decreased TFTs

Normal urinalysis

CLINICAL ALGORITHMS

Normal urinalysis

Evaluate for pericardial constriction

Evaluate for cor pulmonale

Section III

Normal serum albumin

Clear lung fields

Pulmonary hypertension pattern

Check 24-hour urine protein

3.5 g protein

3.5 g protein Check LFTs

Nephrotic syndrome Abnormal LFT LFTs

Myxedema

Normal LFTs Check prealbumin

Evaluate for liver pathology Drug-induced edema

Idiopathic edema

Prealbumin 20 mg/dl

Prealbumin 20 mg/dl

Malnutrition

Capillary leak

Abnormal protein synthesis

FIGURE 3-33 Evaluation of generalized edema. BUN, Blood urea nitrogen; CHF, congestive heart failure; JVP, jugular venous pressure; LFT, liver function tests; TFT, thyroid function tests. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Protein-losing enteropathy

Lymphatic obstruction

Venous obstruction

Negative study

Extrinsic compression

Positive study

Venous thrombosis

Lymphatic obstruction Venous thrombosis Musculoskeletal edema

Venography

Venous insufficiency

Negative study Positive study

Negative study

Positive study

Noninvasive vascular studies or venography

Positive history

FIGURE 3-34 Evaluation of regional edema. CT, Computed tomography; CXR, chest x-ray examination; JVP, jugular venous pressure. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Negative study

Positive study

Doppler study

CXR CT scan

Noninvasive vascular studies or venography Superior vena cava syndrome

Negative history

JVP elevated

Review history

Check JVP

JVP normal

Lower extremity

Upper extremity

Regional edema

ICD-9CM # 250.6

314

EDEMA, REGIONAL

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Edema, Regional

Perform history and physical

Perform CBC, chemistry panel Normal tests

Abnormal tests

Check thyroid function test (TFT) TFT normal

TFT abnormal

Evaluate for infection

Evaluation negative

Evaluation positive

Hypothyroidism Hyperthyroidism

Evaluate for occult malignancy

Evaluation negative

Check nutritional history

Malignancy

Endocarditis Osteomyelitis Tuberculosis Parasitic Fungal

Evaluation positive

Viral prodrome Chronic infection

History negative

History positive

FIGURE 3-35 Evaluation of fatigue. CBC, Complete blood count. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

History and physical normal or nondiagnostic

Anemia Uremia Diabetes mellitus Adrenal insufficiency Hypokalemia Hyponatremia Hepatitis

CLINICAL ALGORITHMS

Fatigue

History and physical abnormal

Toxic exposures Drugs Postconcussion syndrome Congestive heart failure Severe psychologic stress

Heavy metals Carbon monoxide Pesticides Solvents

ICD-9CM # 780.7 Fatigue, general

Depression Chronic viral infection Chronic fatigue syndrome

Nutritional deficiency

Fatigue

Section III

FATIGUE

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FECAL INCONTINENCE ICD-9CM # 787.6 Fecal incontinence in the older adult

Comprehensive evaluation

Dementia?

Functional problems?

Rectal examination

Weak sphincters?

Bowel symptoms

Stool impaction?

Constipation symptoms?

Education and advice

Urgency?

Loose stools?

Evaluate for sphincter weakness

Medical evaluation

No

Yes

Empaction on abdominal X-ray Yes

Prompted or scheduled toileting or controlled evacuation (loperamide plus regular suppositories or enemas)

Facilitate toilet access and safety by multidisciplinary assessment

Sphincter and pelvic muscle exercises plus prn loperamide? biofeedback

Treat constipation diet/fluids/exercise Laxatives for slow transit Suppositories for difficult evacuation Enemas for impaction

Skin care pads Odor control Diet Carer support

FIGURE 3-36 Evaluation of fecal incontinence. (From Tallis RC, Fillit HM [eds]: Brocklehurst’s textbook of geriatric medicine and gerontology, ed 6, London, 2003, Churchill Livingstone.)

BOX 3-1

Clinical Assessment of Fecal Incontinence in Older People

Emphasis in older people is on a structured clinical approach to identify all contributing factors for fecal incontinence History • Duration of fecal incontinence • Frequency of episodes • Type (constant soiling, small amounts, complete bowel movement) • Stool consistency (diarrhea, hard stool) • Unconscious leakage or symptoms of urgency • Constipation symptoms/current laxative use • Systemic illness (confusion, depression, weight loss, anemia) • Antibiotic use General examination • Cognitive and mood assessment • Neurological profile (stroke, autonomic neuropathy, Parkinson’s disease) Toilet access • Evaluate ability to use toilet based on muscle strength, coordination, vision, limb function, and cognition • Place in context of current living environment Specific examination Abdominal inspection for distension and tenderness • Perineal inspection for skin breakdown, dermatitis, surgical scars • Perianal sensation/cutaneous anal reflex • Observe for excessive downward motion of the pelvic floor when asking patient to bear down in the lateral lying position • Digital examination for stool impaction • Digital examination for evaluation of impaired sphincter tone • Anal gaping, and/or easy insertion of finger (internal sphincter) • Reduced squeeze pressure (external sphincter) • Ask patient to strain while sitting on commode and observe for rectal prolapse From Tallis RC, Fillit HM (eds): Brocklehurst’s textbook of geriatric medicine and gerontology, ed 6, London, 2003, Churchill Livingstone.

Check CXR CXR normal

CXR abnormal

Check liver function tests Tests normal

Tests abnormal

Check HIV antibody

Check amylase/ lipase

Antibody negative

Antibody positive

Tests normal

Check drug history

History negative

History positive

Check febrile agglutinins, titers

Drug fever

AIDS AIDS-related complex (ARC)

Hepatitis

Tests elevated

Pancreatitis

Agglutinin negative Perform RBC thick smear

Agglutinin/titer positive

Lyme disease Salmonella Typhus Brucellosis Psittacosis

FIGURE 3-37 Approach to the patient with fever of undetermined origin. AIDS, Acquired immunodeficiency syndrome; ANA, antinuclear antibody; CT, computed tomography; CSR, chest x-ray; ESR, erythrocyte sedimentation rate; GI, gastrointestinal; HIV, human immunodeficiency virus; RBC, red blood cell; UTI, urinary tract infection. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.) Continued on following page

Cultures negative

Tuberculosis Sarcoidosis Lymphoma

CLINICAL ALGORITHMS

Fever of unknown origin

Cultures positive

Smear negative

Smear positive

ICD-9CM # 780.6 Pyrexia of undetermined origin

Babesiosis Malaria

Fever of Undetermined Origin

Section III

Perform blood, urine, throat culture

Endocarditis Sepsis UTI/pyelonephritis Rheumatic fever Sinusitis Tuberculosis

FEVER OF UNDETERMINED ORIGIN

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Smear negative Scan negative Check ESR ESR 50 mm/hr

ESR 50 mm/hr

Perform abdominal/ pelvic CT scan

Check ANA and RF

Scan normal

Scan abnormal

ANA and RF negative

ANA positive

Perform bone marrow aspirate/culture

Endocarditis Pericarditis Myxoma

Marrow normal Perform GI series

Vasculitis

X-ray normal Check viral cultures, titers

Negative

Positive

Cyclic neutropenia Factitious fever Occult malignancy Periodic fever Undiagnosed fever

Epstein-Barr virus Cytomegalovirus Toxoplasmosis

Familial Mediterranean fever

Periarteritis nodosa Aortitis Giant cell arteritis

Tumor Inflammatory bowel disease

Angiogram normal

Angiogram abnormal

X-ray abnormal

Lymphoma Leukemia Tuberculosis

Perform aortic angiography

Echocardiogram normal

Echocardiogram abnormal

Marrow abnormal

Abscess Hepatoma Hypernephroma Lymphoma Pelvic tumor

Perform echocardiogram

RF positive

Rheumatoid arthritis

FIGURE 3-37 (Continued) AIDS, Acquired immunodeficiency syndrome; ANA, antinuclear antibody; CT, computed tomography; CSR, chest x-ray; ESR, erythrocyte sedimentation rate; GI, gastrointestinal; HIV, human immunodeficiency virus; RBC, red blood cell; RF, rheumatoid factor; UTI, urinary tract infection. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

Check bone scan

Scan positive

Metastatic disease Osteomyelitis

Systemic lupus erythematosus

318

FEVER OF UNDETERMINED ORIGIN—cont’d

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Fever of Undetermined Origin

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FRACTURE, BONE ICD-9CM # 829.0 Fracture bone(s) NOS closed 829.1 Fracture bone(s) NOS open

Fracture

Minor trauma Abnormal bone density

Major trauma Normal bone density

Traumatic fracture

Pathologic or low impact fracture

Traction Closed reduction + cast Internal fixation Intramedullary nail plate and screws External fixation Prosthetic replacement

Radiography Bone scan

Solitary lesion

Multiple lesions

Metastatic

Generalized osteopenia

Nonmetastatic

Radiolucent

Radiodense

Prostate

Breast

Nodes

Chest film

Adenopathy

Hematuria Abdominal CT/ MRI or renal ultrasound

Physical examination Radioactive iodine scan

Anemia Elevated ESR SPEP/UEP

Myeloma

Lymphoma

Lung

Kidney

Thyroid

Biopsy

Internal fixation Intramedullary nail or plate and screws ± Methylmethacrylate Prosthetic replacement

Radiotherapy to stabilized lesion

Treatment of multiple lesions: External beam radiotherapy Systemic treatment: Chemotherapy Hormone therapy Radioisotopes Bisphosphonates FIGURE 3-38 Bone fracture. CT, Computed tomography; ESR, erythrocyte sedimentation rate; MRI, magnetic resonance imaging; SPEP, serum protein electrophoresis; UEP, urine electrophoresis. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.) Continued on following page

Bone marrow aspiration

CLINICAL ALGORITHMS

Female: Breast examination Mammogram

Section III

Male: Rectal examination Ultrasonography Acid phosphatase Prostate-specific antigen

Cont’d on next page

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FRACTURE, BONE—cont’d Pathologic or fragility fracture (Cont’d from previous page)

Generalized osteopenia

Solitary lesion

Ca++ PO42Alkaline phosphatase PTH 25-OH-vitamin D 1,25-OH-vitamin D Urinary Ca++

Densitometr y

Consider: Infection Paget’s disease Hemangiomatosis Irradiated bone

Osteoporosis

Parathormone TSH 24-hour urine cortisol Glucose Primar y bone tumor

Benign

Diagnosed on radiography or biopsy

Disuse

Malignant

Endocrinopathy

Biopsy

Idiopathic

Ca++ Vitamin D Hormones

MRI/CT

Biopsy

Cast curette and graft Resect

Wide resection or amputation ± Adjuvant chemotherapy

Traction Closed reduction + cast Internal fixation Intramedullary nail plate and screws External fixation Prosthetic replacement FIGURE 3-38 (Continued)

Osteomalacia

Anemia Elevated ESR SPEP/UEP

Myeloma

Bone marrow aspiration

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GOITER EVALUATION AND MANAGEMENT ICD-9CM # 240.9 240.0 241.9 246.1 242.1 242.2

Goiter, unspecified Goiter, simple Goiter, adenomatous Goiter, congenital Goiter, uninodular with thyrotoxicosis Goiter, multinodular with thyrotoxicosis

History, physical examination (PE) Goiter identified Diffuse or nodular

Free T4↑, TSH↓ Hyperthyroid

Free T4, TSH Ultrasonography (if size and extension unclear)

Therapy: antithyroid drugs, RAI, surgery

Diffuse goiter No obstructive signs

TSH elevated Euthyroid or hypothyroid

T4 therapy Follow free T4, TSH Goiter size by PE

Multinodular goiter, TSH low-normal No obstruction, no dominant nodule

Free T4, TSH normal

T4 therapy Follow T4, TSH Goiter size by PE

Multinodular goiter TSH mid-normal

No obstructive signs Dominant nodule

T4 suppression trial limited to 6 months Frequently no improvement Stop T4 if no size decrease

Significant obstructive signs CT or MRI to determine size and confirm obstruction

Biopsy nodule

Surgery

Benign: T4 suppression limited to 6-12 months Frequently no size decrease Rebiopsy nodule

CLINICAL ALGORITHMS

Minimal obstructive signs

Section III

Free T4, TSH, PE every 6-12 months No T4 therapy

Malignant

Surgery FIGURE 3-39 Evaluation and management of patients with nontoxic diffuse and nodular goiter and undetermined thyroid status. CT, Computed tomography; MRI, magnetic resonance imaging; RAI, radioactive iodine; TSH, thyroid-stimulating hormone. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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GOUT ICD-9CM # 274.9 Diagnostic synovial fluid aspirate Needle aspiration of affected joint

X-ray of affected joint Nondiagnostic aspirate

Suspected gout (initial episode)

Serum uric acid level

Treat

CBC with differential Gram stain and C&S of aspirate

Generally elevated in acute gout but can also be in normal range during acute episode

FIGURE 3-40 Evaluation of suspected gout. CBC, Complete blood count; C&S, culture and sensitivity. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

BOX 3-2

Gout

Diagnostic imaging Best test None Ancillary tests Plain radiograph of affected joint when diagnosis is unclear Lab evaluation Best test Examination of synovial fluid aspirate from affected joint for presence of urate crystals (needle-shaped and birefringent) Ancillary tests Serum uric acid level CBC with differential, ESR if infectious process is suspected Gram stain and C&S of synovial fluid aspirate From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. CBC, Complete blood count; C&S, culture and sensitivity; ESR, erythrocyte sedimentation rate.

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GYNECOMASTIA ICD-9CM # 611.1 Gynecomastia nonpuerperal Gynecomastia

History

Mammography, biopsy

Yes

Physical examination

Asymmetric, fixed unilateral gynecomastia ( discharge) No Medications/drugs?

Yes

Consider trial of discontinuing medications

Yes

Renal disease Liver disease Hyperthyroidism HIV Cushing’s syndrome Refeeding

No Evidence of underlying medical disease? No No

Acute gynecomastia or 5 cm?

Recheck in 6 months

Yes Check T, hCG, LH, estradiol

NL or ↑ T ↑ LH

NL or ↓ T ↓ LH

↑ hCG

↑ Estradiol

All normal

Primary hypogonadism

Androgen insensitivity syndrome

Check prolactin

Ultrasound of testes

Ultrasound of testes

Idiopathic gynecomastia

Secondary hypogonadism

High See Section I “Prolactinoma”

 mass

Testicular germ cell tumor

 mass Chest x-ray CT/MRI of abdomen/ pelvis

 mass

Leydig/ Sertoli cell tumor

 mass

(Adrenal CT)  mass

See Fig. 3-9 (Adrenal Mass)

NL

↑ Extraglandular aromatase activity

FIGURE 3-41 Evaluation of gynecomastia. CT, Computed tomography; hCG, human chorionic gonadotropin; HIV, human immunodeficiency syndrome; LH, luteinizing hormone; MRI, magnetic resonance imaging; NL, normal limits; T, testosterone. (From Noble J: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

CLINICAL ALGORITHMS

NL

Section III

Low T ↑ LH

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HEARING LOSS ICD-9CM # 389.00 Hearing loss, conductive 389.10 Hearing loss, sensorineural Hearing loss

Audiography

Sensorineural hearing loss

Conductive hearing loss

Rule out temporal bone fracture, thrombosis, vasospasm

CT of head with contrast or MRI to rule out external or middle ear pathology

Bilateral

Unilateral

Metabolic evaluation (see ancillary labs)

Auditory brain response (ABR)

Retrocochlear

Cochlear

Acoustic neuroma, meningioma of posterior fossa area, MS

Meniere’s

MRI of brain FIGURE 3-42 Evaluation of hearing loss. CT, Computed tomography; MRI, magnetic resonance imaging. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

BOX 3-3

Hearing Loss

Diagnostic imaging Best test None Ancillary tests CT of head with contrast or MRI with contrast CT of temporal bone without contrast

Lab evaluation Best test None Ancillary tests CBC ALT, AST ANA, VDRL TSH

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging. ALT, Alanine aminotransferase; ANA, antibody to nuclear antigens; AST, angiotension sensitivity test; CBC, complete blood count; CT, computed tomography; TSH, thyroid-stimulating hormone; VDRL, Venereal Disease Research Laboratory test.

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HEARTBURN ICD-9CM # 787.1 Heartburn

History Physical examination

Typical symptoms

Alarm symptoms and signs (dysphagia, weight loss, anemia) warrant immediate endoscopy

Lifestyle modification*

Conventional dose H2RA or proton pump inhibitor

Relief

Therapeutic failure

Step-down treatment

Frequent recurrence

Section III

Infrequent recurrence

Treat again Upper GI endoscopy

Esophagitis

Document reflux: 24-hour pH

More intensive therapy: Proton pump inhibitor

No reflux

Biopsy

Reflux Relief

No Barrett’s esophagus

Barrett’s esophagus

Proton pump inhibitor and surveillance EGD

Failure

Surger y *Weight loss in obese patients, elevation of head of bed Maintenance at night time, and avoidance of caffeine, nicotine, therapy chocolate, peppermint, and any foods that affect lower esophageal sphincter. FIGURE 3-43 Treatment of a patient with heartburn. EGD, Esophagogastroduodenoscopy; GI, gastrointestinal; H2RA, H2 receptor antagonist. (Adapted from Sampliner RE: Heartburn. In Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Normal

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HEMATURIA, ASYMPTOMATIC ICD-9CM # 599.7 Asymptomatic microscopic hematuria in adults

Perform both

Urine cytology Positive

Abdominal ultrasound Negative

Positive for malignancy: urologic consultation

Negative

Positive

Phase-contrast microscopy

Nonglomerular bleeding

Nonsmoker: male 45 yr or female

Masses or other abnormalities further imaging (i.e., CT) as needed; urologic consultation

Dysmorphic RBCs (glomerular bleeding)

Any smoker or male 45 yr

Nephrology consultation; ? renal biopsy

Standard urologic workup (cystoscopy, IVP) Negative Ca:Cr spot urine or peripheral smear for sickling if indicated Positive Manage accordingly

Positive Manage accordingly

Negative Follow-up or nephrology consult; ? renal biopsy

FIGURE 3-44 Suggested algorithm for the evaluation of adult asymptomatic microscopic hematuria. These patients must have no symptoms referable to the hematuria and a negative urinalysis except for red blood cells (RBCs). Adults with gross hematuria require a full urologic evaluation. Ca:Cr, Calcium:creatinine ratio; IVP, intravenous pyelogram. (From Nseyo UO [ed]: Urology for primary care physicians, Philadelphia, 1999, WB Saunders.)

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HEPATITIS, VIRAL ICD-9CM # 070.9 Suspicion of acute viral hepatitis based on: • History, physical examination, epidemiologic situation • Elevated serum aminotransferase activity (ALT/AST)

Obtain viral serologies: • Anti-HAV IgM • HBsAg and Anti-HBc IgM • Anti-HCV (EIA or RIBA)

Anti-HAV IgM positive

Anti-HBc IgM positive with or without HBsAg

Anti-HCV positive

Negative serologies

Diagnosis: Acute hepatitis A infection

Diagnosis: Acute hepatitis B infection

Diagnosis: Acute HCV infection or exacerbation of chronic HCV infection

Consider nonviral etiologies (e.g., ischemia, toxins) or other infectious etiologies (e.g., CMV, EBV)

Suspicion of HDV co-infection based on: • Risk factors (e.g., IVDA) • Clinical signs of severe hepatitis Check anti-HDV

Diagnosis: HBV/HDV co-infection

HBsAg positive with or without abnormal aminotransferase

Recheck anti-HCV in 3-6 months

Diagnosis: Chronic HBV infection

CLINICAL ALGORITHMS

FIGURE 3-45 A flow diagram showing the use of specific serologic tests for the diagnosis of acute viral hepatitis in relation to the clinical and epidemiologic setting. Co-infections and superinfections of chronic hepatitis B or C patients should always be considered in cases that do not fit well with the clinical or serologic picture. CMV, Cytomegalovirus; EBV, Epstein-Barr virus; EIA, enzyme immunoassay; HBV, hepatitis B virus; HCV, hepatitis C virus; HDV, hepatitis D virus; HEV, hepatoencephalomyelitis virus; IVDA, intravenous drug abuse; RIBA, recombinant immunoblot assay. (From Mandell GL: Mandell, Douglas, and Bennett’s principles and practice of infectious diseases, ed 5, New York, 2000, Churchill Livingstone.)

Section III

Anti-HDV positive

Check HBsAg and ALT/ AST in 6-9 months

Consider possibility of HEV infection if recent foreign travel

Hepatomegaly

Perform CT scan

Aminotransferases normal

Measure serum aminotransferases AST 40 U/L ALT 40 U/L

Aminotransferases elevated

Measure viral serologies Viral serologies negative

Viral serologies positive

Perform CT scan No focal parenchymal defects

Focal parenchymal defects

Chronic hepatitis

Acute viral hepatitis

Gallbladder Palpable adjacent mass Feces Thin body habitus Colonic neoplasm Normal variant Riedel’s lobe Asthma Diaphragm displaced downward Emphysema Subdiaphragmatic abscess

CVP normal

Check central venous pressure (CVP)/(JVP)

CVP elevated

Tumor Abscess Cyst Hemangioma

Vascular congestion

Congestive heart failure (CHF) Constrictive pericarditis Tricuspid regurgitation

Primary Metastatic Polycystic disease Echinococcal cysts Congenital hepatic fibrosis

Chronic active hepatitis Chronic persistent hepatitis

Cytomegalovirus (CMV) Epstein-Barr virus (EBV) Hepatitis A Hepatitis B Hepatitis C Hepatitis D Hepatitis E

FIGURE 3-46 Hepatomegaly. ALT, Alanine aminotransferase; AST, aspartate aminotransferase; CT, computed tomography; JVP, jugular venous pressure. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

True hepatic enlargement

Perform history and physical examination

Liver not enlarged

Liver displacement

ICD-9CM # 789.1

328

HEPATOMEGALY

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Hepatomegaly

Perform CT scan No focal parenchymal defects

Focal parenchymal defects

Liver biopsy normal

Perform liver biopsy

Liver biopsy normal

Liver biopsy abnormal

Wilson’s disease Extramedullary hematopoiesis Lymphoma Fatty infiltration Gaucher’s disease Amyloid Granuloma Toxic hepatitis Glycogen infiltration Alpha1-antitrypsin deficiency Iron infiltration Cirrhosis Biliary obstruction Infection Vascular congestion Chronic active hepatitis

Liver normal (reevaluate in 6 months)

Hepatic vein thrombosis Hepatic vein webs Inferior vena cava (IVC) obstruction

Primary Metastatic Polycystic disease Echinococcal cysts Congenital hepatic fibrosis

Venogram normal

Venogram abnormal

Liver normal (reevaluate in 6 months) FIGURE 3-46 (Continued)

Perform liver biopsy

Tumor Abscess Cyst Hemangioma

Perform venogram

CLINICAL ALGORITHMS

CVP normal

Liver biopsy abnormal

Delta hepatitis Wilson’s disease Extramedullary hematopoiesis Lymphoma Fatty infiltration Gaucher’s disease Amyloid Granuloma Toxic hepatitis Glycogen infiltration Alpha1-antitrypsin deficiency Iron infiltration Cirrhosis Biliary obstruction Infection Vascular congestion Chronic active hepatitis

Hepatomegaly

Section III

HEPATOMEGALY—cont’d

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HERPES ZOSTER/POSTHERPETIC NEURALGIA ICD-9CM # 053.9 Zoster

Promote healing Shorten pain duration Reduce duration of postherpetic neuralgia ?Prevent PHN

It is possible that the use of opioids, nortriptyline, or amitriptyline or nerve blocks soon after the development of a acute herpetic pain may help prevent the sensitization of the central nervous system that may lead to persistence of the pain.

Severely symptomatic (all adults) Mildly symptomatic if 50 yrs

50 yrs

Valacyclovir 1 g tid OR Famciclovir 500 mg tid OR Acyclovir 800 mg 5x/day 7-10 days

Consider prednisone 60 mg/day tapered over 21 days

Does not shorten duration of pain May hasten return to premorbid quality of life

PHN

Lidocaine skin patch (Lidoderm) Tylenol  codeine NSAID Nerve blocks Capsaicin

Consider combinations of lidocaine patch, TCAs, gabapentin, opioids at the start or if a single agent fails

Lidocaine patch is reliably effective Relief will be apparent in 1 or 2 weeks

Treatment unsuccessful Choose one or more May be more effective and better tolerated

Sedation, anticholinergic effects, hypotension are a limitation with nortriptyline. Use desipramine if sedation is unacceptable

May be most effective for reducing risk of PHN by attenuating the initiation of central sensitization and limit the structural and functional changes caused by severe acute pain

TCA (tricyclic antidepressants) Nortriptyline or amitriptylene (10 to 20 mg qhs) qhs, with gradual increases until effective or not tolerated 150 mg/day is a high dose Duloxetine 30-60 mg/day is an alternative

Gabapentin Start at low dose, increase to a maximum of 3500 mg per day until there is satisfactory relief or until adverse effects develop

Refractory cases

Refractory cases

Opioids (Controlled-release oxycodone 10 mg q 12 hours, increase dose weekly up to a maximum of 30 mg q 12 hours)

Somnolence, dizziness but usually well tolerated

Pain relief, reduction of allodynia, decreased disability

Persistent refractory cases Pain management center Many other possibilities (e.g., tramadol) Intrathecal methylprednisolone for intractable postherpetic neuralgia of at least one year

FIGURE 3-47 Treatment of herpes zoster and postherpetic neuralgia. PHN, postherpetic neuralgia; TCA, tricyclic antidepressant; NSAID, nonsteroidal antiinflammatory drug. (Adapted from Habif TA: Clinical dermatology, ed 4, St Louis, 2004, Mosby.)

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HYPERCALCEMIA ICD-9CM # 275.24 Hypercalcemia

Evaluate for cancer (consider chest x-ray, PSA, alkaline phosphatase, bone scan)

Positive

Negative

A

Serum parathyroid hormone level

Low • Thiazides • Vitamin A toxicity • Vitamin D toxicity • Milk alkali syndrome • Immobilization • Thyrotoxicosis • Paget’s disease • Granulomatous disorders

High • Primary hyperparathyroidism a. Adenoma b. Hyperplasia, multiple endocrine adenomas c. Recovery from acute tubular necrosis

Section III

Hypercalcemia Treat underlying malignancy Rehydration with normal saline

Plicamycin 25 µg/kg IV; may repeat in 48 hours

Bisphosphonates

B

Etidronate 7.5 mg/kg/day IV for 7 days

Pamidronate

Ca++ 13.5 mg/dl

Ca++ 13.5 mg/dl

60 mg IV over 24 hours

90 mg IV over 24 hours

FIGURE 3-48 A, Evaluation of hypercalcemia. PSA, Prostate-specific antigen. B, Therapy for hypercalcemia. IM, Intramuscularly; IV, intravenously. (From Noble J [ed]: Primary care medicine, ed 3, St Louis, 2001, Mosby.) (From Wachtel TJ, Stein MD: Practical guide to the care of the ambulatory patient, ed 2, St Louis, 2000, Mosby.)

CLINICAL ALGORITHMS

Calcitonin 4-8 µg/kg IM or IV q12h

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HYPERKALEMIA, DIAGNOSTIC APPROACH ICD-9CM # 276.7 Spurious hyperkalemia 1. Tight tourniquet 2. In vitro hemolysis 3. Leukocytosis, thrombocytosis

Hyperkalemia

True hyperkalemia

Impaired entry into intracellular space 1. Insulin deficiency a. Absolute –Type 1 DM b. Relative –Type 2 DM 2. -Blockers 3. Massive K+ intake 4. Hyperkalemic periodic paralysis

Impaired distal NA+ delivery 1. Effective circulating volume depletion 2. Acute renal failure 3. Advanced chronic renal failure (GFR 10 -15 ml/min)

Impaired Na+ reabsorption • Amiloride • Triamterene • Bactrim (trimethoprim)

Redistribution

Shift from intracellular to extracellular space 1. Cell necrosis a. Rhabdomyolysis b. Severe intravascular hemolysis 2. Mineral acidosis 3. Severe exercise 4. Hyperosmolality 5. Succinylcholine 6. Severe digitalis toxicity 7. Resorption of large internal hematoma

Impaired renal K+ excretion (TTKG <8-10, urine K + 200 mEq/day)

Aldosterone resistance • Inherited • Gordon’s syndrome

Impaired K+ secretion in distal nephron

Primary K+ secretory defect • Obstructive uropathy • Tubulointerstitial diseases

Hypoaldosteronism

Hyporeninemic (type IV distal RTA) • Diabetes mellitus • Tubulointerstitial diseases • Nonsteroidal antiinflammatory drugs • Cyclosporine • AIDS

Normal or high renin • Adrenal insufficiency • 21-Hydroxylase deficiency • Angiotensin-converting enzyme inhibitors • Heparin

FIGURE 3-49 Diagnostic approach to hyperkalemia. AIDS, Acquired immunodeficiency syndrome; DM, diabetes mellitus; GFR, glomerular filtration rate; RTA, renal tubular acidosis; TTKG, transtubular potassium gradient. (From Andreoli TE [ed]: Cecil essentials of medicine, ed 4, Philadelphia, 1997, WB Saunders.)

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HYPERKALEMIA, EVALUATION AND TREATMENT ICD-9CM # 276.7 Serum potassium 5.2 mEq/L

Rule out laboratory error 1. Immediately repeat potassium level (obtain heparinized sample) 2. Obtain stat ECG and examine for changes of hyperkalemia a. Tent-shaped and tall T waves b. Flattening of P waves c. AV and intraventricular blocks d. Ventricular arrhythmias

True hyperkalemia associated with ECG changes or clinical manifestations

Place patient on continuous ECG monitoring

Stop all potassium intake (PO, IV)

Administer IV calcium or other antihyperkalemic agents

Stop all medications potentially associated with hyperkalemia (e.g., ACE inhibitors, TMP/SMX, potassiumsparing diuretics)

FIGURE 3-50 Evaluation and treatment of hyperkalemia. ABGs, Arterial blood gases; ACE, angiotensin-converting enzyme; AV, atrioventricular; BUN, blood urea nitrogen; ECG, electrocardiogram; IV, intravenous; PO, oral; TMP/SMX, trimethoprim-sulfamethoxazole. (From Ferri F: Practical guide to the care of the medical patient, ed 6, St Louis, 2004, Mosby.)

CLINICAL ALGORITHMS

Correct underlying cause of hyperkalemia: 1. Dialysis for renal failure 2. Correction of magnesium deficiency 3. Exogenous mineralocorticoids in mineralocorticoid deficiency 4. Correction of acidosis (if present)

Section III

Obtain additional cause laboratory tests: magnesium, calcium, BUN, creatinine, electrolytes, ABGs (in suspected acidosis)

Sweating Diarrhea Vomiting

Extrarenal losses

Hypertonic urine with urinary sodium below 10 mEq/L

Isotonic saline

Isotonic or hypotonic urine with urinary sodium above 20 mEq/L

Glucose Mannitol Urea

Osmotic diuresis

Renal losses

Total body sodium decreased

Sodium and water losses

Hypernatremia

Hypertonic urine with variable urine sodium

Dermal and respirator y insensible losses

Extrarenal losses

Renal losses

Total body sodium normal

Water losses

Urinary sodium and tonicity variable

Diabetes insipidus (DI) Nephrogenic DI

Water replacement

ICD-9CM # 276.0

FIGURE 3-51 Evaluation and treatment of hypernatremia. (From Marx J et al [eds]: Rosen’s emergency medicine: concepts and clinical practice, ed 6, St Louis, 2004, Mosby.)

Water replacement and diuretics

Hypertonic dialysis

Hypertonic sodium infusion

Salt ingestion

Cushing’s syndrome

Primary hyperaldosteronism

Total body sodium increased

Sodium addition

Isotonic or hypertonic urine with urinary sodium above 20 mEq/L

Causes

Diagnosis

Treatment

334

HYPERNATREMIA

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Hypernatremia

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HYPERTENSION, SECONDARY CAUSES ICD-9CM # 401.1 Essential hypertension 401.0 Malignant hypertension due to renal artery stenosis 405.01 Malignant hypertension secondary to renal artery stenosis 437.2 Hypertensive encephalopathy Common clinical features of patients with secondary hypertension • Abrupt onset of hypertension (age 25, age 60) • Episodes of hypertensive crisis • Sudden worsening of blood pressure control • Unresponsiveness to medical prescription (Rx)

Clinical clues to specific diagnosis Upper abdominal bruit • Lateral radiation • Systolic-diastolic • Continuous Hx atherosclerotic disease Worsening renal function during ACE inhibitor Rx

Hypokalemia • Difficulty maintaining serum K with replacement Rx

R/O pheochromocytoma See Section I

R/O hyperaldosteronism See Section I

R/O renovascular hypertension See Section I

Captopril renogram or duplex ultrasonography or MRI angiography

Sleep disturbance • Snoring • Daytime somnolence • Upper body obesity

Diminished or delayed pulses in legs

R/O obstructive sleep apnea See Section I

R/O aortic coarctation

High probability

()

Section III

Low probability

Selective renal angiography

()

Drug Rx close observation

CLINICAL ALGORITHMS

()

Paroxysmal hypertension • Sweating • Palpitations • Headache

() Angioplasty/stenting

(If unsuccessful) Repeat angioplasty drug Rx

Surgical revascularization drug Rx

Drug Rx

FIGURE 3-52 Algorithm for identifying patients for evaluation of secondary causes of hypertension. ACE, Angiotension-converting enzyme; Hx, history; K, potassium; R/O, rule out. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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HYPERTHYROIDISM ICD-9CM # 242.9 Suspected hyperthyroidism

TSH, free T4

Low free, T4, normal/low TSH

Elevated free T4, low TSH

Rule out ingestion of liothyronine

Rule out T3 thyrotoxicosis

Thyroid gland tender

Thyroid gland not enlarged, no nodules

Thyroid gland enlarged

Thyroid scan

Free T3 (elevated)

RAIU scan, serum thyroglobulin level

Rule out thyrotoxicosis factitia (e.g., ingestion of levothyroxine)

Hyperthyroidism

Decreased RAIU, increased serum thyroglobulin

RAIU scan

RAIU scan

Decreased RAIU

Decreased uptake

Low

Elevated

Subacute thyroiditis Autoimmune lymphocytic thyroiditis

Graves‘ disease or toxic nodule FIGURE 3-53 Hyperthyroidism. RAIU, Radioactive iodine uptake; TSH, thyroid-stimulating hormone.

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HYPOCALCEMIA ICD-9CM # 275.41 Hypocalcemia

Check ionized calcium

Low

Normal

Check parathyroid hormone

Low serum albumin

Low (hypoparathyroidism) • • • • •

Hypomagnesemia Renal wasting (gentamicin) Irradiation Surgical Parathyroid infiltration (e.g., amyloid, cancer)

Appropriately elevated • • • • •

Pancreatitis Hyperphosphatemia Hypovitaminosis D Drugs (colchicine, phenytoin) Pseudohypoparathyroidism

FIGURE 3-54 Evaluation of hypocalcemia. (From Wachtel TJ, Stein MD: Practical guide to the care of the ambulatory patient, ed 2, St Louis, 2000, Mosby.)

Section III CLINICAL ALGORITHMS

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HYPOGONADISM ICD-9CM # 256.3 Hypogonadism, female 257.2 Hypogonadism, male LH, FSH, testosterone (T), semen analysis

↓ Sperm count ↓ LH, ↓ FSH, ↓ T

↓ Sperm count LH or FSH, ↓ or NL T

Hypothalamic-pituitary abnormality

Primary testicular abnormality

Measure PRL; MRI of hypothalamicpituitary region

Are testes present in scrotum?

↓ Sperm count NL T & LH; NL or

FSH

Are sperm present?

Yes

No

Seminal fluid fructose No

Yes

hCG stimulation

Size and consistency

T

No T

Cryptorchidism

Anorchia

Small and firm

Postpubertal size, NL or soft

Probable Klinefelter’s syndrome

Acquired primary hypogonadism

Perform karyotype

Present

Absent

Testicular biopsy

Congenital absence of seminal vesicles and vas deferens

Abnormal

NL

Spermatogenic failure

Ductal obstruction

FIGURE 3-55 Laboratory evaluation of hypogonadism. FSH, Follicle-stimulating hormone; hCG, human chorionic gonadotropin; LH, luteinizing hormone; MRI, magnetic resonance imaging; NL, normal; PRL, prolactin; ↑, elevated; ↓, decreased or low. (From Andreoli TE [ed]: Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders.)

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HYPOKALEMIA ICD-9CM # 276.8 Potassium depletion

Urine potassium • Diet: normal Na • UNaV: 100 mEq/day

Low • Diarrhea • Lower GI fistulas

20 mEq/day

20 mEq/day

Extrarenal loss

Renal loss

Serum bicarbonate

Blood pressure

Normal • Cathartics • Profuse sweating

High • Discontinued diuretics or previous vomiting • Gastric fistula

Elevated

Normal

Plasma renin

Serum bicarbonate

Low • RTA

Low

High

Urine chloride

Plasma aldosterone

Low • Cushing’s syndrome • Ingested mineralocorticoid • Congenital adrenal hyperplasia

10 mEq/day • Vomiting

10 mEq/day • Diuretics • Bartter’s syndrome • Normotensive hyperaldosteronism • Mg depletion • Extreme K depletion

FIGURE 3-56 Diagnostic approach to hypokalemia. Because renal potassium wasting may improve during sodium restriction, diminished potassium excretion is indicative of extrarenal loss only when the diet (and therefore the urine) is rich in sodium. GI, Gastrointestinal; HBP, high blood pressure; RTA, renal tubular acidosis; UNaV, urinary sodium volume. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Elevated • Primary aldosteronism

Section III

High • Malignant HBP • Renovascular HBP • Renin-secreting tumor

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HYPONATREMIA ICD-9CM # 276.1

Diagnosis

Causes

Hyponatremia

Treatment

340

Deficit of total body water and larger sodium deficit

Excess total body water

Excess of body sodium with greater excess of total body water

ECF volume contraction

Mild ECF volume expansion

ECF volume expansion

Renal losses

Extrarenal losses

Diuretics Renal tubular acidosis Salt-losing nephropathies Mineralocorticoid deficiency

GI fluid losses Third spacing

Urinary sodium above 20 mEq/L

Urinary sodium below 10 mEq/L

Isotonic saline

SIADH Hypothyroidism Glucocorticoid deficiency

Nephrotic syndrome Congestive heart failure Cirrhosis

Renal failure

Urinary sodium above 20 mEq/L

Urinary sodium below 10 mEq/L

Urinary sodium above 20 mEq/L

Water restriction

Water restriction

FIGURE 3-57 Evaluation and treatment of asymptomatic, mild hyponatremia. ECF, Extracellular fluid; GI, gastrointestinal; SIADH, syndrome of inappropriate secretion of antidiuretic hormone. (From Marx J et al [eds]: Rosen’s emergency medicine: concepts and clinical practice, ed 5, St Louis, 2002, Mosby.)

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HYPOPHOSPHATEMIA ICD-9CM # 275.3 Serum phosphate 2 mg/dl

Exclude the presence of: Glucose infusions Respiratory alkalosis

If absent, measure urinary phosphate excretion

Low (100 mg/day)

GI losses

Internal redistribution

High (100 mg/day)

Evaluate for the presence of glycosuria, uricosuria, aminoaciduria, and bicarbonaturia

Present: Fanconi syndrome

Absent

Section III

Analyze serum calcium

• Primary hyperparathyroidism • Hypercalcemia of malignancy

• • • • •

Secondary hyperparathyroidism Oncogenic osteomalacia Recovery from renal failure Vitamin D–resistant rickets Familial hypophosphatemia

FIGURE 3-58 Diagnostic workup of hypophosphatemia. GI, Gastrointestinal. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Low or normal

High

Hypotension

FIGURE 3-59 Saunders.)

Check tissue perfusion Decreased perfusion

Perfusion normal

Normal volume

Drug history negative

Check intravascular volume

Volume normal or increased

Decreased volume

NOS chronic iatrogenic orthostatic or postural

Cardiovascular hypotension

Heart failure Valvular dysfunction Pericardial tamponade Arrhythmia Pulmonary embolus

Idiopathic postural hypotension Shy-Drager syndrome Spinal cord lesions Peripheral neuropathy

Hypovolemia Sepsis Neuropathic Anaphylactic Drug-induced Addisonian crisis Metabolic acidosis

Autonomic dysfunction

Vasovagal hypotension

Check drug history

Drug history positive

Antihypertensives Nitrates Phenothiazines Minor tranquilizers Tricyclic antidepressants

Ascites Pleural effusions Edema

Hypotension, Hypotension, Hypotension, Hypotension,

Hypotension. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB

Nonorthostatic hypotension

Check for orthostatic changes

Orthostatic hypotension only

Check intravascular volume

Decreased volume

Blood loss Dehydration Third spacing of fluid Adrenal insufficiency

ICD-9CM # 458.9 458.1 458.2 458.0

342

HYPOTENSION

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Hypotension

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HYPOTHYROIDISM ICD-9CM # 244 Suspected hypothyroidism

TSH, free T4

Normal/low TSH, low free T4

Elevated TSH, normal/low free T4

Elevated TSH, low free T4

Rule out secondary hypothyroidism (hypopituitarism)

Rule out early hypothyroidism, Hashimoto‘s thyroiditis, subacute thyroiditis, autoimmune lymphocytic thyroiditis, recovery from severe illness

Primary hypothyroidism

MRI of pituitary gland FIGURE 3-60 Hypothyroidism. MRI, Magnetic resonance imaging; TSH, thyroid-stimulating hormone.

Section III CLINICAL ALGORITHMS

Treatment

Diagnosis certain

Radionuclide scan

Chronic hepatocellular disease (cirrhosis)

Treatment

Diagnosis certain

Liver biopsy

Laparoscopy

Diagnosis uncertain

Liver biopsy

Diagnosis uncertain Diagnosis or uncertain chronic course

Hepatitis antigen and antibody tests Cardiac evaluation

Acute hepatocellular disease

Blood and urine tests Liver function tests

Treatment

Liver biopsy

Ducts patent

ERCP

Ducts not dilated

Bone evaluation

Bone origin

Surgery or endoscopic stenting

Ducts obstructed

Treatment

Liver biopsy

Ducts patent

Liver biopsy

Blood pool scan or MRI

Solitary filling defect—? hemangioma*

Treatment

Follow, no treatment *LFTs usually normal

Directed liver biopsy

Positive Partial Diagnosis Diagnosis bile duct certain uncertain Negative obstruction

ERCP

Multiple Normal filling defects

Echography or CT scan

Liver origin

Fractionate alkaline phosphatase or 5-nucleotidase

Infiltrative disease Partial bile duct obstruction Isolated alkaline phosphatase increase

Percutaneous cholangiography

Ducts dilated

Echography or CT scan

Cholestasis (intra- and extrahepatic)

FIGURE 3-61 Evaluation of jaundice and hepatobiliary disease. BSP, Bromsulphalein; CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; LFTs, liver function tests; MRI, magnetic resonance imaging. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

Further hematologic tests Effect of fasting on bilirubin, BSP test

85% indirect Hemolysis Gilbert’s syndrome Both direct and indirect Dubin-Johnson and Rotor’s syndrome

Isolated bilirubin elevation

History, physical examination

ICD-9CM # 782.4 Jaundice NOS 277.4 Bilirubin excretion disorders 576.8 Jaundice, obstructive

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JAUNDICE AND HEPATOBILIARY DISEASE

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Jaundice and Hepatobiliary Disease

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JOINT EFFUSION ICD-9CM # 719.0 Joint effusion

Arthrocentesis and analysis of fluid

Suspected gonococcal arthritis

Suspected Lyme disease

WBC 200 -10,000 PMNs 50%

Elevated total protein concentration

Lyme titer

Elevated WBC 10,000 PMNs 50% Glucose level 40 mg/dl over serum glucose level

Examination for crystals under polarized light

Noninflammatory Calcium pyrophosphate

Thayer-Martin cultures

Rule out inflammatory or septic arthritis Monosodium urate, needle shaped, strongly birefringent

Rule out infectious process

Confirm by negative Gram stain and negative C&S

Pseudogout

FIGURE 3-62

Joint effusion. C&S, Culture and sensitivity; WBC, white blood cell count.

Gout

Section III

Gram stain, C&S

CLINICAL ALGORITHMS

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JOINT SWELLING ICD-9CM # 719.00 Joint pain and swelling

Synovial Fluid Studies Cell counts Crystals Gram stain Culture

Inflammatory WBC 2,000/mm3

Noninflammatory WBC 2,000/mm3

Other Clinical Features History Physical findings Laboratory tests Imaging studies

Inflammatory Arthritis Differential: Septic arthritis Rheumatoid arthritis Systemic lupus erythematosus Seronegative spondyloarthropathies Crystal-induced arthritis Lyme arthritis

Noninflammatory Arthritis Differential: Osteoarthritis Meniscal and/or ligament tears Avascular necrosis

FIGURE 3-63 Diagnostic approach for swollen joints. WBC, White blood cell count. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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LEG ULCER ICD-9CM # 440.23 Ulcer, lower limb, arteriosclerotic 707.1 Ulcer, lower limb, chronic 707.1 Ulcer, lower limb, neurogenic 707.9 Ulcer, non-healing 707.0 Pressure ulcer Leg ulcer History Physical examination

History of rest pain Cool distal extremity

Chronic edema Varicosities Pigment changes

Normal vasculature

Arterial ulcer Venous ulcer Skin biopsy Arteriography

Decreased cutaneous sensation

Consider: Diabetes mellitus Exogenous neurotoxins Alcoholism Sarcoidosis Leprosy Syphilis

Consider: Venous occlusion Thrombophlebitis

Erythema Purulent exudate

Chronic ulceration Elevated edges

Skin biopsy Culture of exudate

Skin biopsy

Infectious ulcer

Consider: Bacterial Fungal Mycobacterial Treponemal Viral Parasitic

Neoplastic ulcer

Consider: Squamous cell carcinoma Basal cell carcinoma Sarcoma Lymphoma Metastasis

History of trauma

Traumatic ulcer

Consider: Burn/heat Frostbite Pressure Postradiation status Insect bite Self-induced/factitial

FIGURE 3-64 Leg ulcer. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

CLINICAL ALGORITHMS

Neurotrophic ulcer

fibrinogen test Doppler ultrasound Plethysmography Venography

Section III

Consider: Emboli/thrombi Arteriosclerosis obliterans Thromboangiitis obliterans Hypertensive leg ulcer Raynaud’s disease/livedo reticularis Vasculitis/pyoderma gangrenosum Sickle cell disease

125I-labeled

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LIVER FUNCTION TEST ELEVATIONS ICD-9CM # 573.9 Elevated LFTs

Rule out lab error

Repeat LFTs

Rule out NASH

Rule out drug-induced

Rule out alcohol abuse

Weight loss if obese

Discontinue potentially offending agents (e.g., statins, NSAIDs, niacin, acetaminophen)

Stop all alcohol intake

Repeat LFTs in 2–6 weeks

Persistent elevation of LFTs

Ultrasound of liver

Normal

Rule out alpha-1 antitrypsin deficiency

Serum protein IEP

Rule out primary biliary cirrhosis

Antimitochondrial Ab

Rule out Wilson‘s disease

Ceruloplasmin level, serum copper level

Normal results on additional testing but persistent LFT elevation

Abnormal or equivocal

Rule out autoimmune hepatitis

Rule out infectious hepatitis

ANA, smooth muscle Ab, LKM-1 Ab

Hepatitis panel

Rule out hemochromatosis

Transferrin saturation, ferritin level

Abnormal results

Liver biopsy Repeat LFT in 3 months

Worsening LFT results

Liver biopsy FIGURE 3-65 Liver function test elevations. Ab, Antibody; ANA, antibody to nuclear antigens; CT, computed tomography; IEP, immuno-electrophoresis; LFT, liver function test; LKM, liver-kidney microsome; NSAIDs, nonsteroidal antiinflammatory drugs.

CT of liver

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LYMPHADENOPATHY, AXILLARY ICD-9CM # 785.6 Axillary adenopathy

None of the above

Skin lesion on trunk or arms

Painful axillary adenopathy

Biopsy if persistent adenopathy

Biopsy of skin lesion (rule out melanoma), lymph node biopsy

Rule out infected hair follicle, cellulitis

CBC with differential, blood cultures 2

FIGURE 3-66

Breast exam/mammography

Breast biopsy if abnormal findings

Positive history of animal contact with scratch, dermal, or eye lesion

Rule out CSD

Lymph node biopsy, CBC with differential, culture of lymphatic aspirate, CSD skin test

Lymphadenopathy, axillary. CBC, Complete blood count; CSD, cat-scratch disease.

Section III CLINICAL ALGORITHMS

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LYMPHADENOPATHY, CERVICAL ICD-9CM # 785.6 Cervical lymphadenopathy

Painless

Suspicious skin lesion

Biopsy of skin lesion, lymph node biopsy

Painful

Lymph node biopsy if persistent

Positive history of animal contact with scratch, dermal, or eye lesion

CBC with differential, viral titers, throat C&S

Rule out CSD

Lymph node biopsy, CBC with differential, culture of lymphatic aspirate, CSD skin test FIGURE 3-67 Lymphadenopathy, cervical. CBC, Complete blood count; C&S, culture and sensitivity; CSD, cat-scratch disease.

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LYMPHADENOPATHY, GENERALIZED ICD-9CM # 785.6 Lymphadenopathy, unknown etiology Recent lymph node(s) enlargement (0.5 cm) with no obvious cause by history or physical examination

Follow-up in 2-4 weeks ( CBC, ESR, ALT, creatinine, chest x-ray, serology)

If suspicious for malignancy, pursue

If it persists, increases, or new node develops

If CBC suggests CLL, “consider” bone marrow aspiration, test for lymphocyte markers, abdominal ultrasound or CT, lymph node biopsy; treat if indicated

Inflammatory: evaluate etiology

Granulomas: evaluate etiology

If it resolves, follow up periodically

If CBC is not diagnostic, consider biopsy of most accessible node (try to avoid inguinal node biopsy)

Lymphoma

Hodgkin’s disease

Metastatic lesion: evaluate etiology

Nondiagnostic: repeat biopsy if markers show no monoclonality

Non-Hodgkin’s lymphoma (NHL)

Treatment FIGURE 3-68 Workup of lymphadenopathy. ALT, Alanine aminotransferase; CBC, complete blood count; CLL, chronic lymphocytic leukemia; CT, computed tomography; ESR, erythrocyte sedimentation rate. (Modified from Noble J [ed]: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

CLINICAL ALGORITHMS

Histologic subclassification Immunologic subclassification for NHL Clinical staging Pathologic staging where appropriate

Section III

Further evaluation

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MALABSORPTION ICD-9CM # 579.9 Suspected malabsorption

CBC, serum B12 level, RBC folate, albumin, cholesterol, serum carotene, PT, serum calcium

Suspicious

Normal

Sudan III stain of stool x2

Diagnosis unlikely

Positive for fat droplets

Negative

D-Xylose

Diagnosis unlikely

test

Normal

Rule out pancreatic insufficiency (secretin test, bentiromide test, serum trypsin–like immunoreactivity test) or response to pancreatic extract

Abnormal

Small-bowel biopsy and jejunal aspirate to rule out mucosal lesion

FIGURE 3-69 Suspected malabsorption. Ab, Antibody; CBC, complete blood count; IgA, immunoglobulin A; PT, prothrombin time; RBC, red blood count. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

BOX 3-4

Antigliadin Ab, lgA endomysial Ab

Positive

Negative

Treat for celiac disease

Carbon 14–xylose breath test or therapeutic trial of doxycycline to rule out bacterial overgrowth

Malabsorption, Suspected

Diagnostic imaging Best test Small-bowel series Ancillary test CT of pancreas with IV contrast Lab evaluation Best test Biopsy of small bowel

Ancillary tests Albumin, total protein ALT, AST, PT Serum lytes, BUN, creatinine Sudan III stain of stool for fecal leukocytes CBC, RBC folate, serum iron, serum carotene, cholesterol, serum calcium Hydrogen 14-C xylose breath test D-Xylose test, secretin test Quantitative fecal test Antigliadin antibody, IgA endomysial antibody

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. ALT, Alanine aminotransferase; AST, aspartate aminotransferase; BUN, blood urea nitrogen; CBC, complete blood count; CT, computed tomography; IgA, immunoglobulin A; IV, intravenous; PT, prothrombin time; RBC, red blood count.

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MULTIPLE MYELOMA ICD-9CM # 203.0 Suspected multiple myeloma (pallor, bone pain, purpura)

Serum protein immunoelectrophoresis

CBC and x-ray of painful areas

M-spike not present

M-spike

Serum and urine immunoelectrophoresis reveals light chains

Serum immunoelectrophoresis positive for marked increase in IgG, IgE, or IgD

Light-chain myeloma

Bone marrow confirms diagnosis of myeloma

FIGURE 3-70 Multiple myeloma. CBC, Complete blood count; Ig, immunoglobulin.

Section III CLINICAL ALGORITHMS

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MURMUR, DIASTOLIC ICD-9CM # 785.2 Murmur heart Diastolic murmur

Increases with inspiration

Does not increase with inspiration

Right-sided murmur: Tricuspid stenosis Pulmonic regurgitation

Left-sided murmur: Mitral stenosis Aortic regurgitation Austin Flint murmur

Murmur quality and location

Murmur quality and location

Low-pitched Left lower sternal border

High-pitched Left upper sternal border

Tricuspid stenosis Rheumatic usually with associated mitral stenosis

Pulmonic regurgitation

Pulmonary hypertension present

Pulmonary hypertension absent

Graham Steell murmur

Pulmonary regurgitation Infective endocarditis Congenital

Low-pitched Apical

High-pitched Sternal border

Uncertain

Mitral stenosis: Rheumatic Calcific Other: Left atrial myxoma

Aortic regurgitation: Valvular Aortic root dilation

Associated findings Effect of amyl nitrate

Decrease

Increase

Aortic regurgitation Austin Flint murmur

Mitral stenosis

Acute

Chronic

Murmur low pitched, shorter Normal pulse pressure Peripheral signs absent Pulmonary edema— acute and early Normal LV size

Murmur high-pitched, longer Wide pulse pressure Peripheral signs present Pulmonary edema—late Increased LV size

FIGURE 3-71 Diastolic murmur. LV, Left ventricle. (From Greene HL, Johnson WP, Lemke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

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MURMUR, SYSTOLIC ICD-9CM # 785.2 Murmur heart Systolic murmur

Increases with inspiration

Does not increase with inspiration

Right-sided murmur: Tricuspid regurgitation Pulmonic outflow/ stenosis

Left-sided murmur: AS HCM MR VSD MVP

Ejection

Regurgitant

Pulmonic outflow

Tricuspid regurgitation: Rheumatic Nonrheumatic (e.g., carcinoid, Ebstein’s) Right ventricular dilation

Other heart sounds

Ejection sound

Ejection

Regurgitant

AS HCM

MR VSD MVP

Maneuvers

Maneuvers

No ejection sound

No fixed S2

Atrial septal defect

Disappears on standing or Valsalva

MR VSD Midsystolic click(s)

Intensity varies with maneuvers

AS HCM Innocent murmur

ECG Chest film MVP

Innocent pulmonic flow murmur

Increase with Valsalva Decrease with squatting Increase with standing

Does not increase with Valsalva Does not decrease with squatting Does not increase with standing

HCM

AS Aortic sclerosis Innocent flow murmur

Other heart sounds Murmur characteristics

FIGURE 3-72 Systolic murmur. AS, Aortic stenosis; ECG, electrocardiogram; HCM, hypertrophic cardiomyopathy; MR, mitral regurgitation; MVP, mitral valve prolapse; PS, pulmonary stenosis; VSD, ventricular septal defect. (From Greene HL, Johnson WP, Lemke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

AS: Ejection sound Loud, harsh Right upper sternal border Left ventricular hypertrophy

Innocent flow murmur: No associated abnormalities Soft, early systolic Left sternal border

CLINICAL ALGORITHMS

Fixed S2

No change

Maneuvers

Section III

Timing varies with Valsalva or squatting

PS

Uncertain

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MUSCLE CRAMPS AND ACHES ICD-9CM # 729.82 Muscle cramps and aches

At rest

With exercise

Mainly legs

Focal, buttocks and thighs, calves

Exercising muscles

Normal neurologic examination

Neurologic examination Elevated (or normal) CPK

Normal serum enzyme levels (CPK)

Normal

Adult

Abnormal

Serum enzyme levels (CPK) Exclude: Dehydration Hemodialysis Hypocalcemia Hypokalemia Hyper- or hypothyroidism Uremia Medication use (e.g., statins, fibrates, diuretics)

Ordinary (benign) cramps

Elevated

Claudication

Exclude: Peripheral vascular disease

Normal

EMG

Abnormal

Forearm ischemic exercise test

Muscle biopsy Biochemical studies

Metabolic myopathy

Disappears with rest

Investigate and treat accordingly

Normal

Observe

Repeat studies normal

Exclude: Neuromuscular disorder (e.g., motor neuron disease, polymyositis, limb-girdle muscular dystrophy, myotonic dystrophy) Peripheral neuropathy Nerve root compression

If ankle-brachial index test is normal, consider spinal stenosis

Refer to neurologist

Ordinar y (benign) cramps

FIGURE 3-73 Evaluation of muscle cramps and aches. CPK, Creatine phosphokinase; EMG, electromyography. (Adapted from Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Primary myopathy

Neurologic exam normal

Tests normal

CK/aldolase elevated

CK/aldolase normal

Sed rate elevated

Sed rate normal

Review drug history

Negative drug history

Positive drug history

Perform electromyography (EMG)

EMG normal

Exercise-induced Sampling error Muscle biopsy normal

Perform electromyography (EMG)

EMG normal

EMG abnormal

Endogenous depressive psychosis

Muscular dystrophies Congenital myopathies Amyloidosis Metabolic storage disease

Carnitine deficiency

Adult acid maltase deficiency

Muscular dystrophy Sarcoidosis Muscle biopsy abnormal

Rheumatoid arthritis Systemic lupus erythematosus Polyarteritis nodosa Dermatomyositis/polymyositis Amyloidosis Sarcoidosis Polymyalgia rheumatica (PMR) Corticosteroids (steroid myopathy) D-penicillamine Colchicine Chloroquine Clofibrate Cimetidine Statins

EMG normal

EMG abnormal

Perform muscle biopsy

Polymyositis Dermatomyositis

Progressive myopathy Seizures Hyperthermia Crush injury Extreme exertion Alcoholic myopathy

ICD-9CM # 782.9

EMG abnormal

Familial periodic paralysis Diabetes mellitus Hypo/hyperthyroidism Hypo/hyperkalemia Hypercalcemia Hypermagnesemia Hypophosphatemia Hyperparathyroidism Vitamin D deficiency Renal tubular acidosis Cushing’s syndrome Rhabdomyolysis McArdle’s syndrome Familial myoglobinuria

Perform electromyography (EMG)

Measure sedimentation rate (M 5 mm/hr; F 12 mm/hr)

Measure serum creatine kinase (CK) and aldolase

Check serum glucose, electrolytes, Ca, Mg, phosphate, FT4I, TSH level, and myoglobinuria

Systemic symptoms absent

Staphylococcal Streptococcal Salmonella Clostridial Mycobacterium leprae

Metabolic abnormality

Parasitic

Bacterial

Tests abnormal

Infection

Check for systemic symptoms, fever

Systemic symptoms and fever present

Viral

Poliomyelitis Rabies Coxsackievirus Influenza Rubella HIV (AIDS) Epstein-Barr virus (EBV)

CLINICAL ALGORITHMS

FIGURE 3-74 Muscle weakness. AIDS, Acquired immunodeficiency syndrome; EBV, Epstein-Barr virus; F, female; HIV, human immunodeficiency virus; M, male. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

Muscle weakness

Perform history and physical, including neurologic exam

Neurologic exam abnormal

Neurogenic myopathy

Guillain-Barré syndrome Porphyria Myasthenia gravis Eaton-Lambert syndrome Amyotrophic lateral sclerosis

Section III

MUSCLE WEAKNESS

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Muscle Weakness 357

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MYELODYSPLASTIC SYNDROMES ICD-9CM # 238.7 1. Determine whether degree of dysplasia is 10% in any of the following lineages: erythroid/myeloid/megakaryocytic

Yes

No

2. Are bone marrow blasts 20%?

Consider cytogenetics and/or other studies (e.g., X-linked, RFLP, oncogene mutations, calcitonin gene hypermethylation) for clonality

Yes

20% 30%

No

30%

3. Determine number of peripheral blood monocytes

1 109/L

Acute leukemia

1 109/L

4. Stratify further in terms of BM blasts

5% 20% blasts

5% blasts

5. Determine number of ringed sideroblasts (RS)

Refractory anemia with excess blasts in transformation

Chronic myelomonocytic leukemia

Refractory anemia with excess blasts

15% RS

5% RS

Refractory cytopenia (anemia)

Refractory anemia with ringed sideroblasts

FIGURE 3-75 Myelodysplastic syndromes. BM blasts, Bone marrow blastocyst; RFLP, restriction fragment length polymorphism. (From Abeloff MD: Clinical oncology, ed 2, New York, 2000, Churchill Livingstone.)

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MYOCARDIAL ISCHEMIA, SUSPECTED ICD-9CM # 411.89 Myocardial Ischemia, acute without MI 414.8 Myocardial Ischemia, chronic 411.1 Angina, stable 413 Angina pectoris Suspected myocardial ischemia

Nonacute presentation

Acute presentation

Radionuclide stress test or stress echocardiogram

Cardiac troponins, CK-MB

2, ECG

Negative

Positive

Seek alternate diagnosis

Cardiac catheterization

Negative

Diagnostic

Radionuclide stress test or stress echocardiogram

FIGURE 3-76 Myocardial ischemia, suspected. CK-MB, Myocardial muscle creatine kinase isoenzyme; ECG, electrocardiogram.

Section III CLINICAL ALGORITHMS

Sarcoidosis, other granulomatous diseases, lymphomas [high serum 1,25(OH)2vitamin D3] Hyperthyroidism (high T3, T4) Myeloma (osteoclast activating factor) Malignant tumor PTH-like peptide in hypercalcemia of malignancy; prostaglandins

Primary hyperparathyroidism

FIGURE 3-77 Evaluation of patients with suspected nephrolithiasis (flank pain, ureteral colic, hematuria, fever). AMP, Adenosine monophosphate; PTH, parathyroid hormone. (From Stein JH [ed]; Internal medicine, ed 5, St Louis, 1998, Mosby.)

Normal or low serum PTH and urine cyclic AMP

High serum PTH High urine cyclic AMP

Hypercalcemia

Distal renal tubular acidosis

Idiopathic hypercalciuria Renal Ca leak [may include medullary sponge kidney; secondary hyperparathyroidism and activation of 1,25(OH)2-vitamin D3 synthesis] Renal P leak [activation of 1,25(OH)2-vitamin D3 synthesis; probably indirect] Absorptive hypercalciuria [mediated via increased 1,25(OH)2-vitamin D3stimulated intestinal Ca absorption or by augmented gut Ca absorption independent of vitamin D]

Metabolic acidosis (venous blood pH 7.34 serum HCO3 22 mEq/L serum CI 108 mEq/L urine pH always 6.0 low urine citrate)

Hyperuricosuria and Ca stone syndrome (some also have hypercalciuria)

Idiopathic calcium stone disease Habitually low fluid intake and thus concentrated urine ? Deficiency of inhibitor of crystal nucleation or growth ? Presence of promoter of crystal nucleation or growth Primary hyperoxaluria Acquired hyperoxaluria Small bowel disease Gluttony for oxalaterich food Ascorbic acid abuse

Purine gluttony Idiopathic uric acid stone Diarrheal diseases

Normal serum uric acid

Normal urinary uric acid and oxalate excretion

Gout Malignancy

Hyperuricemia

Uric acid stone (confirm by analysis)

Uric acid crystalluria Urine pH 5.5 Concentrated urine

Hyperoxaluria (urine oxalate 45 mg/day)

Normal urine Ca

Cystine stone (confirm by analysis)

Cystine crystalline Acid urine Positive cyanide nitroprusside test

Hyperuricosuria (urine uric acid 800 mg/day in men; 750 mg/day in women)

Hypocitraturia (200 mg/day)

Hypercalciuria (300 mg Ca/day or 4 mg Ca/kg/day) No acidosis

Infection stone caused by urease-producing bacilli Evaluate mechanism for urinary infection Search for underlying metabolic cause of stone that became secondarily infected

Struvite-carbonate apatite stone (confirm by analysis)

Struvite-apatite cr ystalluria Urine pH 7.5 Pyuria and bacilluria

Normal serum Ca

Calcium oxalate-apatite stone (confirm by analysis)

Calcium oxalate or apatite cr ystalluria

Radiolucent stone (also consider tumor of renal pelvis, blood clot, sloughed renal papilla)

Examination of sediment from urine specimen immediately after voiding Obtain plain abdominal radiograph, ultrasonogram, and intravenous urogram

Radiodense stone

(1) (2)

ICD-9CM # 592.9 Urinary calculus

360

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Nephrolithiasis

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NEUTROPENIA ICD-9CM # 288.0 START

Complete blood count with differential and platelet count

Neutrophil count

Fever (38° C) or signs of infection?

1 109/L

Yes

No

Hospitalize, obtain cultures, and consider empiric antibiotic Rx

Neutrophil count 2 109/L  1 109/L

No

Selective neutropenia?

Bi- or pancytopenia

Yes Examine peripheral blood smear and check MCV

Taking drugs? (see Table 3-8) No

Yes No

Discontinue suspect drug. Response?

Oval macrocytes or high MCV?

History of recurrent infections? No

No

Toxin exposure?

Family history of chronic neutropenia?

Toxins? Drugs? No

Obtain serum B12 and folate levels. Low?

No

No

No

Yes

Idiopathic, congenital, pseudoneutropenia

No

Bone marrow examination

Hypocellular

Aplastic anemia AIDS Drug-related Toxic Immunologically mediated Immune injury Cytotoxic T cells (T) Antibody-mediated (Ab) Both T and Ab Toxin-mediated injury Certain viral infections Mycobacterial infections Myelodysplasia Paroxysmal nocturnal hemoglobinuria Hereditary neutropenia syndromes

Yes

Normal or increased cellularity

Normal morphology? No Acute leukemia Myelodysplasia Lymphoma Hairy cell leukemia AIDS Carcinoma Fibrosis Immune injury Megaloblastic (B12/folate deficiency)

Yes Neutrophil destruction Neutrophil utilization Infection Trauma Sequestration Tissue necrosis Hypersplenism

FIGURE 3-78 A practical algorithm for the evaluation of patients with neutropenia. The fundamental diagnostic principle is that for patients with severe neutropenia or for those with bicytopenia or pancytopenia, bone marrow examination will likely be necessary unless the following diagnoses are made: (1) a nutritional (folate or vitamin B12) deficiency or (2) drug- or toxin-induced neutropenia in a patient whose neutropenia resolves after discontinuation of the offending agent. AIDS, Acquired immunodeficiency syndrome; MCV, mean corpuscular volume; RBC, red blood cell. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

CLINICAL ALGORITHMS

Reconsider B12/folate deficiency. Order RBC folate, methylmalonate, homocysteine. Abnormal?

Yes

Section III

Chronic inflammatory or autoimmune disease?

Yes B12/folate deficiency

Hypersegmented neutrophils?

No

No

Yes

Yes

Discontinue. Response?

Stop exposure.

Yes Familial neutropenia

Yes

No

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NUTRITIONAL SUPPORT ICD-9CM # 269.9 Nutritional assessment

Nutritional support indicated

Gut can be used safely

Yes

No

Oral intake 2/3 of required

Parenteral nutrition

Yes

No Tube feedings

2 weeks

2 weeks

Nasoenteric tube

Tube enterostomy

Increased risk for aspiration

Increased risk for aspiration

Yes

No

Yes

No

Nasoduodenal

Nasogastric

Jejunostomy

Gastrostomy

FIGURE 3-79 Decision approach for the type and route of nutritional support. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

BOX 3-5

Indications for the Use of Enteral Nutrition in Adult Medical Patients

Protein-energy malnutrition with anticipated significantly decreased oral intake for at least 7 days Anticipated significantly decreased oral intake for 10 days Severe dysphagia Massive small bowel resection (used in combination with total parenteral nutrition) Low-output (500 mL/day) enterocutaneous fistula From Goldman L, Ausiello D (eds): Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.

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PAIN CONTROL ICD-9CM # not available Source of Pain

Peripheral or central nervous system lesion

First-line drugs Try singly, then in combination: Gabapentin or Tricyclic antidepressant or Opioid (mu agonists or tramadol)

Second-line drugs If first-line drugs are ineffective, seek consultation or try: carbamazepine, lamotrigine, paroxetine, citalopram, venlafaxine, clonidine, dextromethorphan

Focal lesions in bone, joint, viscera, or other tissues

Multisomatoform disorder (includes many patients with fibromyalgia, irritable bowel, chronic fatigue, idiopathic low back pain, headache, or other syndromes that include multiple unexplained symptoms)

Nonopioid: acetaminophen or NSAID Establish whether effective or not If effective, dose to adequate relief or maximum safe dose

SSRI antidepressants Cognitive-behavioral therapy Aerobic exercise

Mu opioid agonist or tramadol Rescue doses if additional analgesia is needed Establish whether effective or not

Section III

If opioid partly effective, increase until relief adequate or side effects limit dose.

For dose-limiting opioid side effects, try one of three approaches

Add medications to treat side effects

For doses equivalent to 90 mg/day of oral morphine, convert entire dose For doses equivalent to 90 mg/day of oral morphine, convert 50% of dose at a time For switches to the slowly eliminated methadone, be aware of the possibility of overdosing due to drug accumulation on days 2-3.

Sedation: methylphenidate Nausea: scopolamine, hydroxyzine, phenothiazine, 5HT3 blocker? Constipation: bulk  stimulant

Adjust dose timing and route to lower peaks

FIGURE 3-80 Algorithm for the treatment of pain. COX, Cyclooxygenase; NSAID, nonsteroidal anti-inflammatory drug; SSRI, selective serotonin reuptake inhibitor. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

CLINICAL ALGORITHMS

Switch to different opioid

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PANCREATIC MASS ICD-9CM # 789.36 Suspected pancreatic cancer

Helical CT scan with thin cuts through pancreas

No mass

Mass

EUS

Metastatic

No mass

Evaluate for other causes of abdominal complaints Consider ERCP or MRI if suspicion still high

ERCP with cytology (if obstruction is present) or biopsy (metastatic site preferred)

Mass

Potentially resectable

No metastases

Unresectable

EUS FNA*

Unresectable Biliary obstruction

No biliary obstruction Resection or FNA*

Resectable

FNA ERCP with cytology

Negative

Percutaneous biopsy of mass

Negative

Consider EUS with FNA of mass FIGURE 3-81 Diagnostic algorithm for pancreatic cancer. Intraoperative fine-needle aspiration (FNA) if found inoperable during surgery. CT, Computed tomographic scan; ERCP, endoscopic retrograde cholangiopancreatography; EUS, endoscopic ultrasonography; MRI, magnetic resonance imaging. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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PARKINSON’S DISEASE ICD-9CM # 332.0 Idiopathic Parkinson’s disease 332.1 Parkinson’s disease, secondary Parkinson’s disease

Nonpharmacologic treatment

Pharmacologic treatment

Consider neuroprotective agent(s) (selegiline)

Functional impairment? Additional drugs: Anticholinergics Amantadine

Cognitively normal

L-DOPA

DA agonists

(CR)

Yes

No

Cognitively impaired or very old (80 yr old)

COMT inhibitors

Drug modification to improve efficacy and reduce side effects

Surgery

FIGURE 3-82 Diagrammatic representation of a therapeutic approach to patients with parkinsonism. COMT, Catechol-O-methyl transferase; CR, controlled release; DA, dopamine. (Adapted from Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

Section III

Combination therapy

CLINICAL ALGORITHMS

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PELVIC MASS ICD-9CM # 789.39 Pelvic mass

Ultrasound (US)

Simple cyst 6 cm

Premenopausal

Cyst 6-8 cm or size 6 cm with any equivocal US findings

Suspicious for malignancy

Nonovarian lesion

CA-125

CA-125

Appropriate evaluation

Gynecologic referral Consideration of laparoscopy

Gynecologic oncology referral Laparotomy

Postmenopausal

Repeat examination and US in 1-3 months

Resolution of cyst

Persistent cyst

? CA-125

Oral contraceptive pill

Repeat US FIGURE 3-83 Approach to the patient with a pelvic mass. US, Ultrasound. (From Carlson KJ et al: Primary care of women, ed 2, St Louis, 2002, Mosby.)

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PERIPHERAL NEUROPATHY ICD-9CM # 356.9 Peripheral nerve neuropathy 355.10 Lower extremity neuropathy 354.11 Upper extremity neuropathy Patient with peripheral neuropathy History Physical examination

Mononeuropathy

Multifocal mononeuropathy

NCS EMG

Focal

Symmetric polyneuropathy

NCS EMG Nerve biopsy Widespread Axonal

Consider: Compression Entrapment Ischemia Trauma Infiltration

Consider: Diabetes Vasculitis Leprosy Sarcoid

Demyelinating conduction block

Multifocal variant of CIDP

NCS EMG Blood tests ( nerve biopsy)

Section III

Axonal

Demyelinating

Subacute

Chronic

Consider: Porphyria Tick paralysis Toxins

Consider: Metabolic disorder (diabetes, uremia) Toxin Drugs Nutritional deficiency Vasculitis Infection Dysproteinemia Amyloid Malignancy

Consider: Metabolic disorder Toxin Drugs Nutritional deficiency Vasculitis Infection Dysproteinemia Amyloid Malignancy Hereditary condition

Chronic uniform slowing

Variable course Nonuniform slowing

Consider: Hereditary condition Refsum’s disease Metachromatic leukodystrophy

Acute

Relapsing or chronic

Consider: Guillain-Barré syndrome Diphtheria

Consider: CIDP Dysproteinemia Myeloma Lead toxicity

FIGURE 3-84 Approach to the patient with peripheral neuropathy. CIDP, Chronic inflammatory demyelinating polyradioneuropathy; EMG, electromyogram; NCS, nerve conduction studies. (From Greene HL, Johnson WP, Lemcke DL: Decision making in medicine, ed 2, St Louis, 1988, Mosby.)

CLINICAL ALGORITHMS

Acute

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PLEURAL SPACE FLUID ICD-9CM # 511.9 Pleural effusion, unspecified Pleural space fluid

Diagnostic thoracentesis

Clear, yellow; LDH low; pH 7.30; glucose normal; protein low

Clear or turbid; LDH high (200 IU) pH 7.30; glucose low (60 mg/100 ml); protein high (3.0 g/100 ml)

Frank pus; LDH high (1500 IU); pH low (7.20); glucose low (40 mg/100 ml); protein high (3.5 g/100 ml)

Transudate

Exudate

Empyema

Many RBC or bloody

Few WBC or at least 1000/mm 3

PARAPNEUMONIC Many WBC, 1000/mm 3, culture

Etiology: malignant neoplasm, pulmonar y infarction, chest wall trauma

Etiology: rheumatoid arthritis, pancreatitis: uremia, drug reaction

Etiology: bacterial pneumonia, abscess of lung, tuberculosis, M. pneumoniae fungal disease

Etiology: cirrhosis, congestive heart failure, SLE, albumindeficiency conditions, myxedema

Treat underlying condition; thoracentesis may be needed to relieve respiratory distress

Many WBC, 20,000/mm3, culture unless antibiotics given previously

Etiology: bacterial pneumonia, abscess of lung, penetrating chest wounds, tuberculosis, fungal disease, postthoracotomy, malignant neoplasm

Treat with appropriate antibiotics; therapeutic thoracentesis as necessary

Treat with appropriate antibiotics; drainage using large-bore chest tube and water seal; surgical intervention as necessary

FIGURE 3-85 Evaluation, common etiologies, and management of pleural effusion and empyema. LDH, Lactate dehydrogenase; RBC, red blood cells; SLE, systemic lupus erythematosus; WBC, white blood cells. (From Kassirer J [ed]: Current therapy in adult medicine, ed 4, St Louis, 1998, Mosby.)

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PREOPERATIVE EVALUATION, PATIENT WITH CORONARY HEART DISEASE

ICD-9CM # 411.89 Coronary insufficiency, acute 411.8 Coronary insufficiency, chronic 411.1 Coronary insufficiency or intermediate syndrome Assess stability of ischemic symptoms

Unstable coronary syndrome

Stable ischemic symptoms

Unstable angina Accelerated angina Postinfarction angina

Assess cardiac functional status

High risk

Stable class I or II angina (see Table 3-3)

Functional status not apparent from history

Stable class III or IV angina (see Table 3-3)

Low risk

High risk

Patient unable to exercise

Perform exercise treadmill

Assess clinical predictors and multifactorial index score

Unable to achieve 75% MPHR or ischemia on ECG

Low risk

High risk

No clinical predictors and low-risk index score

1 or 2 clinical predictors or intermediate index score

2 clinical predictors or high-risk index score

Low risk

Intermediate risk

High risk

Proceed with dipyridamolethallium scintigraphy or dobutamine echocardiogram

Normal test

Abnormal test

Low risk

High risk

FIGURE 3-86 Preoperative evaluation of patients with known or suspected coronary artery disease. (From Goldman L, Braunwald E [eds]: Primary cardiology, Philadelphia, 1998, WB Saunders.)

TABLE 3-3

New York Heart Association Functional Classification

Class I Class II

No limitation Slight limitation

Class III

Marked limitation

Class IV

Inability to carry on any physical activity

From Ferri FF: Ferri’s clinical advisor, St Louis, 2006, Mosby.

Ordinary physical activity does not cause symptoms Comfortable at rest Ordinary physical activity causes symptoms Comfortable at rest Less than ordinary activity causes symptoms Symptoms present at rest

CLINICAL ALGORITHMS

Able to achieve 75% MPHR without ischemia

Section III

Patient able to exercise

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PROSTATE CANCER ICD-9CM # 185 Age 70

Is PSA elevation genuine?

Age 70

Excellent for age family longevity

Review diagnostic criteria

Transrectal ultrasound (TRUS) with biopsy

Challenged very poor performance status

Appropriate for age

Obstructive symptoms No

Yes

Establish histological diagnosis; TRUS and biopsy

Refer to urologist: ?TUR(P)

Very rarely in presence of overt tumor (PSA, DRE) but in absence of histological confirmation. Treat to alleviate clinical distress (bone pain, upper tract obstruction)

Diagnosis established

Biopsy positive

Skeletal status?

Bone scan

Negative

Histological grade: Gleason score: Treatment opinions:

Refer to urologist for consideration of radical curative therapy

Positive

Well 2-4

Mod. 5-7

Poor 8-10

Well Mod. Poor 2-4 5-7 8-10

WW Consider surgery, radiation therapy

WW Consider surgery, radiation therapy, HM

HM

HM

Vigilant follow-up

FIGURE 3-87 Assessment and treatment of a patient with prostate cancer suspected on the grounds of a digital rectal exam and PSA. WW, Watchful waiting; HM, hormonal manipulation; PSA, prostate specific antigen. (Modified from Tallis RC, Fillit HM [eds]: Brocklehurst’s textbook of geriatric medicine and gerontology, ed 6, London, 2003, Churchill Livingstone.)

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PROSTATIC HYPERPLASIA, BENIGN ICD-9CM # 600 Benign prostatic hyperplasia Patient presenting with voiding symptoms

AUA symptom score (AUASS) (completed before history and physical examination) (see Section I)

History

Indications for immediate urologic intervention: Urinary retention, renal failure, gross hematuria, recurrent urinary tract infections, bladder stones

Medications (diuretic, anticholinergic, α-adrenergic: side effects) Previous instrumentation or transurethral procedure; prior pelvic surgery Neurologic disease/diabetes

PHYSICAL EXAMINATION • Focused GU examination: back, abdomen, external genitalia • Focused neurologic examination • DRE PERTINENT DIAGNOSTIC STUDIES

Moderate symptoms (moderate bothersomeness) AUASS13

Severe symptoms (severe bothersomeness) AUASS19

TREATMENT OPTIONS*

* Treatment options in the absence of (1) indications for immediate urologic consultation/intervention and/or (2) abnormal DRE or PSA.

MEDICAL TREATMENT • Initiate α-blocker with or without finasteride for moderate symptoms and moderate prostate size (30 g) • Initiate finasteride with or without α-blocker for severe symptoms and large prostate size (40 g)

Failures

SURGERY Symptoms associated with indication(s) for immediate urologic consultation/ intervention: TUIP vs. TURP vs. open prostatectomy vs. minimally invasive (i.e., laser, VaporTrode, hyperthermia, microwave)

FIGURE 3-88 Critical pathway for patients with benign prostatic hypertrophy. AUA, American Urological Association; DRE, digital rectal examination; GU, genitourinary; PSA, prostate-specific antigen; TUIP, transurethral incision of the prostate; TURP, transurethral resection of the prostate. (From Nseyo UO [ed]: Urology for primary care physicians, Philadelphia, 1999, WB Saunders.)

CLINICAL ALGORITHMS

OBSERVATION or low-dose α-blocker, particularly if symptoms are mild to moderate

Section III

Mild symptoms (mild bothersomeness) AUASS7

• Renal function • Urinalysis • Serum PSA • Urine cytology, if urinary symptoms are mostly irritative

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PROTEINURIA ICD-9CM # 791.0 Proteinuria History Physical examination

Urine dipstick test

24-hour urine protein 150 mg/day

24-hour urine protein 150 mg/day

Pathologic

Probably normal

Urine protein electrophoresis 24-hour collection

3.5 g/day Nephrotic syndrome

3.5 g/day Exclude: Congestive heart failure Hypertension Nephritis Fever Acute tubular necrosis

No glucose intolerance

Glucose intolerance

Consider: Diabetic glomerulosclerosis

Family history of kidney disease

Abnormal proteins

No family history of kidney disease

Exclude: Causes of nephrotic syndrome

Consider: Alport's disease Fabry's disease

Exclude: Multiple myeloma

Consider: Amyloidosis Light-chain disease

Consider: Transient proteinuria Benign persistent proteinuria Orthostatic proteinuria

Examine serology

Elevated ANA

Low C3, C4

Positive ANCA

Positive anti-GBM antibody

Serologic examination negative

Consider: Lupus GN

Consider: Lupus GN Postinfectious GN Cryoglobulinemia Membranoproliferative GN

Consider: Systemic vasculitis Wegener's granulomatosis

Consider: Goodpasture's syndrome

Consider: Focal sclerosis Minimal change disease Drugs

Hepatitis B or HIV antigenemia Consider: Hepatitis AIDS-related nephropathy

Renal biopsy

Treat underlying disease Prevent secondary progression

FIGURE 3-89 Proteinuria. AIDS, Acquired immunodeficiency syndrome; ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic autoantibody; anti-GBM, anti–glomerular basement membrane; GN, glomerulonephritis. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Consider: Biliary cirrhosis Drug-related condition Biliary obstruction

Consider: Polycythemia vera Leukemia Myeloma Iron deficiency

Consider: Hodgkin’s disease Carcinoma

Consider: Thyrotoxicosis Hypothyroidism

Diabetes mellitus

Consider: Psychogenic pruritus Drug reaction Carcinoma

Normal

Abnormal Abnormal

Abnormal

Renal failure

Complete laborator y profile

BUN/creatinine Thyroid function tests

Glucose tolerance test

Diagnostic tests

No skin lesions

ICD-9CM # 698.9 Pruritus NOS

Pruritus, Generalized

Section III CLINICAL ALGORITHMS

FIGURE 3-90 Evaluation of generalized pruritus. BUN, Blood urea nitrogen. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Abnormal

Abnormal

Abnormal

Liver function panel

Hemogram

Diagnostic tests: Skin biopsy Scabies preparation Urticaria workup

Consider: Xerosis (dry skin) Atopic dermatitis Scabies Dermatitis herpetiformis Drug eruption Fiberglass dermatitis Urticaria Mycosis fungoides

Skin lesions

History Physical examination

Generalized pruritus

Chest radiography

PRURITUS, GENERALIZED

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PULMONARY EMBOLISM ICD-9CM # 415.1 Suspected PE

Baseline abnormalities on chest x-ray

Normal baseline chest x-ray

Lung scan

Inconclusive

Diagnostic

Spiral CT of chest

Diagnostic

Inconclusive

Compression ultrasound of lower extremities and plasma D-dimer

Positive

Negative

Pulmonary angiogram if clinical suspicion is high FIGURE 3-91 Pulmonary embolism. CT, Computed tomography; PE, pulmonary embolism. (From Ferri FF: Ferri’s clinical advisor, St Louis, 2006, Mosby.)

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PULMONARY NODULE ICD-9CM # 518.89 Newly diagnosed pulmonary nodule on chest x-ray

Obtain old films if available

Age 40, never smoked, nodule with benign features of calcifications

New or enlarging nodule in comparison with prior chest x-ray

Nodule unchanged for 2 years

CT of lung

Follow

Multiple nodules

Single nodule

Evaluate for metastatic carcinoma Follow with CT of chest every 6 months for 2 years

Biopsy if indicated

Smoker, * age 40

Never smoked

PET scan

PET scan

PET scan

Resect

Negative

Positive

Nodule 1 cm

Nodule 1 cm

Resect or biopsy

Repeat CT in 3–6 month intervals

Resect

Negative

Negative nodule 1cm

Negative nodule 1 cm

Positive

Negative

Resect or biopsy

Follow with CT scan every 4 months 2 years

Biopsy if enlarging

Biopsy

Repeat CT scan of chest in 3–6 months Nodule enlarging

* Current or previous smoker

Resect if enlarging Resect

FIGURE 3-92 Pulmonary nodule. CT, Computed tomography; PET, positron emission tomography. (From Ferri FF: Ferri’s clinical advisor, St Louis, 2006, Mosby.)

CLINICAL ALGORITHMS

Positive

Section III

Smoker, * age 40

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RED EYE, ACUTE ICD-9CM # 379.93 History of trauma Yes

No

Obvious open globe

Fluorescein test

No Fluorescein test

Positive • Corneal abrasion • Corneal ulcer

Negative or variable • Subconjunctival hemorrhage • Traumatic iritis • Hyphema • Ruptured globe

Negative or punctate staining only

Positive • Corneal ulcer • Corneal erosion • HSV keratitis

Response to topical anesthesia

No relief Pupillary status Normal or miotic • Angle-closure glaucoma

• Iritis • Scleritis

Pain relieved (or no pain) • Conjunctivitis • Blepharitis • UV keratitis • Conjunctival foreign body • Dry eye • Subconjunctival hemorrhage • Episcleritis • Contact lens overwear syndrome

FIGURE 3-93 Algorithm showing diagnostic procedure for the acute red eye. HSV, Herpes simplex virus; UV, ultraviolet. (From Auerbach PS: Wilderness medicine, ed 4, St Louis, 2001, Mosby.)

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RENAL FAILURE, ACUTE ICD-9CM # 584.9 Acute renal failure, unspecified Acute renal failure

Prerenal 1. Absolute decrease in effective blood volume Hemorrhage Skin losses (burns, sweating) Gastrointestinal losses (diarrhea, vomiting) Renal losses (diuretics, glycosuria) Fluid pooling (peritonitis, burns) 2. Relative decrease in blood volume (ineffective arterial volume) Congestive heart failure Sepsis Anaphylaxis Liver failure 3. Arterial occlusion Bilateral thromboembolism Thromboembolism of solitary kidney

Intrinsic renal

Vascular Examples: vasculitis, malignant hypertension, microscopic polyarteritis

Acute glomerulonephritis Examples: postinfectious glomerulonephritis, anti–basement membrane antibody disease

Postrenal 1. Obstruction of collecting system Bladder outlet obstruction Bilateral ureteral obstruction (unusual) Ureteral obstruction in a solitary kidney

Acute interstitial nephritis Examples: drug-associated acute interstitial nephritis (methicillin)

Acute tubular necrosis

Ischemic Hypotension

FIGURE 3-94 Causes of acute renal failure. (From Andreoli TE [ed]: Cecil essentials of medicine, ed 4, Philadelphia, 1997, WB Saunders.)

CLINICAL ALGORITHMS

Endogenous 1. Intratubular pigments (hemoglobinuria, myoglobinuria) 2. Intratubular proteins (myeloma) 3. Intratubular crystals (uric acid, oxalate)

Section III

Exogenous Nephrotoxins: 1. Antibiotics (gentamicin, kanamycin, neomycin, amikacin, tobramycin, streptomycin, amphotericin B) 2. Iodinated contrast agents 3. Metals (mercur y, cisplatin) 4. Solvents (carbon tetrachloride, ethylene glycol)

Nephrotoxic

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RENAL MASS ICD-9CM # 593.9 Symptoms of hematuria

Cystic Smooth wall No internal echoes

Renal ultrasound

Observe

Solid/complex Internal echoes Irregular wall

Negative CT number Fat density Angiomyolipoma

CT scan

Observe

Complex mass No contrast enhancement Indeterminate

Avascular Inconclusive

Needle aspiration

Renal arteriogram

Mass not identified (confirmed with CT scan)

Solid Contrast enhancement Vascular tumor

Suspected caval thrombus

Surgery

MRI

Neovascularity

Surgery

Malignant cells

Surgery FIGURE 3-95 Evaluation of a patient with a renal mass. CT, Computed tomography; MRI, magnetic resonance imaging. (Modified from Williams RD: Tumors of the kidney, ureter, and bladder. In Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

History of fever, voice change

Visualize pharnyx, larynx, glottis

Epiglottitis Abscess Foreign body Bacterial tracheitis

Tracheomalacia Extrinsic/intrinsic mass

Abn

Foreign body Extrinsic/intrinsic mass

Abn

WNL

Asthma Bronchiolitis

Yes

Pneumonia Bronchiolitis Empyema Effusion Pulmonary embolism Pulmonary edema

Abn

No

Response to β agonist

WNL

Chest x-ray ABG, if needed

No

Wheezing

WNL

No

Treat with diuretics

Yes

Effusion CHF Pulmonar y edema Elevated brain natriuretic peptide (BNP)

Abn

Chest x-ray

Asthma Psychogenic Bronchiectasis Foreign body CHF Pneumonia

Yes

No

Pulmonary embolism Other: Acidosis Salicylism Shock ↓ Fio2

No

Heart, liver, renal disease

No

Acute onset

Pneumothorax Musculoskeletal Contusion Hemothorax Flail chest Yes Rib fracture

Abn

Yes Trauma

Chest x-ray

Yes

No

ICD-9CM # 786.09 Respiratory distress NOS 518.82 Respiratory distress, acute

Respiratory Distress

Section III CLINICAL ALGORITHMS

FIGURE 3-96 Respiratory distress. ABG, Arterial blood gas; Abn, abnormal; CHF, congestive heart failure; WNL, within normal limits. (Adapted from Barkin RM, Rosen P: Emergency pediatrics, St Louis, 1999, Mosby.)

*Do not visualize without immediate capability of airway intervention. If epiglottitis is suspected, procedure should be performed under controlled conditions, often in the operating room.

Yes

Stridor

Inspir/expir film

Asthma Bronchiolitis

WNL

Visualize trachea (elective bronchoscopy)

Foreign body Neck injur y Cord paralysis Extrinsic/intrinsic mass Laryngomalacia

WNL

No

Abn

No

Yes

Wheezing

Abn

Visualize pharynx, larynx, glottis*

Yes

RESPIRATORY DISTRESS

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RHINORRHEA ICD-9CM # 478.1 Rhinorrhea

Histor y Physical examination

Symptoms 7-14 days

Chronic symptoms

Transillumination History of recurrent sinus infections

Skin testing

Opaque

Clear

Negative

Positive Acute sinusitis

Viral rhinitis

Chronic sinusitis

Allergic rhinitis

Symptomatic treatment Vasomotor rhinitis

Normal host

Rhinitis medicamentosa

Miscellaneous

Immunocompromised: Nosocomial Evidence of complications

Treat empirically

Improves

No improvement

Consider: Radiography CT Antral puncture FIGURE 3-97 Approach to a patient with rhinorrhea. CT, Computed tomography. (From Noble J [ed]: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

Psychotherapy

Nocturnal erections present by history or testing

Abnormal

Psychotherapy

Diagnose and treat medical disorder Discontinue offending medication

Psychiatric history

Determine FSH, LH, prolactin, testosterone level (male)

Decreased semen volume or Change in menstrual cycle

Consider: Medication effect Drug, alcohol abuse Chronic illness Acute illness Situational stress Fatigue Depression

Decreased libido

Normal

Organic cause

Nocturnal erections not present by strain gauge or stamp test

Consider: Endocrine disorders Penile diseases Neurologic disorders Vascular disorders Drug effect

Impotence

Interview both partners Examine partners separately

Sexual dysfunction disclosed

Psychotherapy

History of sexual abuse or rape

Sexual therapy

No history of sexual abuse or rape

Educate patient: Anatomy Sexual response Self-exploration

Orgasmic dysfunction

Female dysfunction

Diagnose and treat medical disorder

Consider: Vaginitis Cystitis Trigonitis Leiomyomatous uterus Adnexal mass

Dyspareunia

ICD-9CM # 309.2 Sexual disorder (psychosexual) V41.7 Sexual function problem

Sexual Dysfunction

CLINICAL ALGORITHMS

FIGURE 3-98 Evaluation of sexual dysfunction. FSH, Follicle-stimulating hormone; LH, luteinizing hormone. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Counseling and supportive therapy

Increase coital frequency Squeeze technique

Consider: Situational stress Fatigue Sexual expectations

Ejaculation disorder

Male dysfunction

Complete history and physical examination Obtain sexual history

Sexual dysfunction

Section III

SEXUAL DYSFUNCTION

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SJÖGREN’S SYNDROME ICD-9CM # 710.2 Dry eyes for  3 mo, recurrent sensation of sand or gravel in the eyes, or use of tear substitutes  3x per day

or

Daily feeling of dry mouth for  3 mo, recurrent or persistent swollen salivary glands, or use of liquids to aid in swallowing dry food

Negative

Positive Positive Shirmer's test ( 5 mm in 5 min), or a Rose Bengal score of  4 according to van Bijsterveld scoring system

No SS

Negative

Positive

Focus score  1 in a minor salivary gland biopsy

Focus score  1 in a minor salivary gland biopsy

Negative

Positive

Negative

Positive

No SS

Positive result in one of the following tests: salivary scintigraphy, parotid sialography or unstimulated salivary flow ( 1.5 ml in 15 min)

Antibodies to Ro(SS-A) or LA(SS-B), antinuclear antibodies, or rheumatoid factors

SS

Negative

Positive

Negative

Positive

No SS

SS

Positive result in one of the following tests: salivary scintigraphy, parotid sialography or unstimulated salivary flow ( 1.5 ml in 15 min)

SS

Negative

Positive

No SS

SS

FIGURE 3-99 Algorithm for the diagnosis of Sjögren’s syndrome. (From Tzoufas AG, Moutsopoulos HM: Sjögren’s syndrome. In Klippel JH, Dieppe P [eds]: Rheumatology, ed 2, London, 1998, Mosby, with permission.)

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SLEEP DISORDERS ICD-9CM # 780.50 Sleep disorder, unspecified cause Sleep disturbance History

Insomnia

Hypersomnia

Sleep-associated affective and behavioral disturbance

See Algorithm B

See Algorithm C Duration 3 weeks

Normal sleep hygiene

Duration 3 weeks

Poor sleep hygiene

Consider: Situational disorder Work shift change Jet lag syndrome

Advise on rules of better sleep hygiene Spontaneous resolution Reassurance Possible short course of hypnotics

Psychophysiologic condition

Psychiatric consultation and therapy

Not stress related

Alcohol or drug use

Abstention Counseling

Stress reduction Relaxation techniques Stimulus control Daily exercise Deconditioning

Consider: Restless legs syndrome Periodic movements of sleep Central sleep apnea Chronic respiratory failure Alpha delta sleep pattern

PSG to confirm diagnosis

Chronic pain syndrome

Identify source

Treat

PSG may reassure and exclude other possibilities FIGURE 3-100 A, Patient with sleep disturbance. MSLT, Multiple sleep latency tests; PSG, polysomnography. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.) Continued on following page

CLINICAL ALGORITHMS

Psychiatric

Section III

Stress related

A

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SLEEP DISORDERS—cont’d Hypersomnia

History of prolonged nocturnal sleep

PSG MSLTs

Associated with disrupted nocturnal sleep

Associated with insomnia

Insomnia primary cause

Idiopathic narcolepsy

Delayed sleep phase syndrome

See Algorithm A

PSG MSLTs

Chronotherapy

Stimulants Tricyclics

B

Idiopathic CNS hypersomnolence

Kleine-Levin syndrome (rare)

Menstrualassociated syndrome

Stimulants

Resolves Refer to SDC

Estrogen

Sleep drunkenness or Long sleeper syndrome

PSG Respiratory and limb EMG monitoring

Obstructive sleep apnea syndrome

Periodic movements of sleep

Continuous positive airway pressure

Benzodiazepines Opiates Dopaminergic drugs

Associated with normal nocturnal sleep

Reassurance Refer to SDC Intercurrent medical illness

Psychiatric illness

Medication side effects

Identify

Psychiatric consultation

Substitute other medication

Treat

Consider: PSG with MSLTs FIGURE 3-100 (Continued) B, Hypersomnia. CNS, Central nervous system; EMG, electromyelogram; MSLTs, multiple sleep latency tests; PSG, polysomnography; SDC, sleep disorders clinic.

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SLEEP DISORDERS—cont’d Sleep-associated affective and behavioral disturbance

No recollection of event

Nocturnal seizures

All-night EEG with video

Anticonvulsants

Night terrors

Good recollection of event

Nightmares

Panic attacks

Rapid eye movement behavioral disorders

Reassurance Tricyclics Monoamine oxidase inhibitors

Tricyclics

Clonazepam

C

PSG with infrared video

Reassurance Psychotherapy Benzodiazepines Tricyclics

FIGURE 3-100 (Continued) C, Sleep-associated affective and behavioral disturbance. EEG, Electroencephalogram; PSG, polysomnography.

Section III CLINICAL ALGORITHMS

Confirm Spleen scan or CT

Asymptomatic Follow

FIGURE 3-101 Clinical approach to patient with splenomegaly. CT, Computed tomography. (From Stein JH [ed]: Internal medicine, ed 5, St Louis, 1998, Mosby.)

Symptomatic Splenectomy for diagnosis

Bone marrow nondiagnostic, cultures negative

Bone marrow aspiration, biopsy, and cultures Hematologic conditions Chronic fungal and mycobacterial infections Gaucher’s disease Amyloidosis

With lymphadenopathy See Fig. 3-68

Results negative or equivocal

Examine peripheral blood smear Hematologic malignancies Nonmalignant hematologic disease Parasitemia

Immunologic causes excluded

Evaluate for immunologic disorders Systemic lupus erythematosus Rheumatoid arthritis Felty’s syndrome

Splenic cyst or displacement of normalsized spleen excluded

Exclude Portal hypertension Congestive heart failure Subacute bacterial endocarditis

Without lymphadenopathy

Splenomegaly

ICD-9CM # 789.2 Splenomegaly, unspecified 289.51 Splenomegaly, chronic congestive 759.0 Splenomegaly, congenital 789.2 Splenomegaly, unknown etiology

386

SPLENOMEGALY

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Splenomegaly

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SPONDYLOSIS, CERVICAL ICD-9CM # 721.0 Cervical spondylosis

Neck pain/headache

Root syndrome

Cord syndrome

Hard pillow, soft collar at night; heat and massage

Hard pillow, soft collar full-time 2-3 wks; heat and massage

Collar plus MRI

Improved

No better

Improved

No better

Stop

Radiograph of cervical spine

Stop

MRI

Mild pathology

Severe pathology

Collar

Consult surgeon

Reevaluate Physical therapy; medicate No better

Sleep in collar

Consult surgeon

FIGURE 3-102 Algorithm for the treatment of cervical spondylosis. MRI, Magnetic resonance imaging. (From Ronthal M, Rachlin JR: Cervical spondylosis. In Johnson RT, Griffin JW [eds]: Current therapy in neurologic disease, ed 5, St Louis, 1997, Mosby.)

Section III

Improved

CLINICAL ALGORITHMS

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STROKE ICD-9CM # 436 Brief history and examination (complete 10 min after arrival)

Consistent with stroke

Inconsistent with stroke

Initial procedures Alert radiology (head CT scan) Blood studies • CBC • Platelets • Coagulation studies • Chemistry and liver panel • Finger stick glucose Insert access IV

Continue evaluation

Obtain brain CT (preliminary interpretation 45 min after arrival)

Hemorrhage

No new lesions Early infarct signs present

Subarachnoid

Parenchymal

Appropriate for tPA? (3 hr)

No

Yes

No

RBCs in multiple tubes Xanthochromia

Continue evaluation

Identify etiology and start prophylaxis

Yes Blood pressure in acceptable range (185/100)?

Able to lower BP?

No

Yes Yes Administer tPA (1 hr after arrival)

No

Identify etiology and start prophylaxis FIGURE 3-103 Algorithm for the emergency evaluation of a patient with suspected stroke. BP, Blood pressure; CBC, complete blood count; CT, computed tomography; RBCs, red blood cells; tPA, tissue plasminogen activator. (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

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SYNCOPE ICD-9CM # 720.2 Syncope

Carotid sinus massage

Detailed history, physical exam

ECG, labs

Diagnostic (e.g., vasovagal syncope, orthostatic hypotension)

Suspected CNS

Suspected cardiac

Carotid Doppler, CT of head, EEG

Echocardiography

24-Hour Holter monitor

Exercise treadmill test

EPS studies

Negative

Tilt-table testing

Syncope

Diagnostic imaging Best test None. Diagnostic imaging should be guided by history and physical exam Ancillary tests Echocardiography is useful in patients with a heart murmur to r/o aortic stenosis, hypertrophic cardiomyopathy, or atrial myxoma If seizure is suspected, CT of head and EEG is indicated Spiral CT of chest or ventilation/perfusion scan if PE is suspected

Lab evaluation Best test None Ancillary tests Routine blood tests rarely yield diagnostically useful information and should be done only when specifically suggested by history and physical exam CBC, lytes, BUN, creatinine Serum calcium, magnesium ABGs ECG Cardiac troponins, isoenzymes if history of chest pain before syncope Toxicology screen in selected patients Cardiac stress test Electrophysiologic (EPS) studies

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. ABG, Arterial blood gas; BUN, blood urea nitrogen; CBC, complete blood count; CT, computed tomography; ECG, electrocardiograph; EEG, electroencephalograph.

CLINICAL ALGORITHMS

BOX 3-6

Section III

FIGURE 3-104 Syncope evaluation. CNS, Central nervous system; CT, computed tomography; ECG, electrocardiograph; EEG, electroencephalograph; EPS, electrophysiologic. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

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TACHYCARDIA, NARROW COMPLEX ICD-9CM # 427.2 Paroxysmal tachycardia 427.0 Supraventricular paroxysmal tachycardia 427.89 Atrial tachycardia Narrow complex tachycardia Unstable DC shock Stable Regular rhythm

No Atrial fibrillation Atrial flutter with variable block Multifocal atrial tachycardia (MAT)

Yes PSVT: AV node reentry AV reentry Atrial tachycardia ? Sinus tachycardia

Adenosine 6-18 mg IVP MAT

Afibrillation/flutter

Ineffective Verapamil 5-10 mg IVP

IV Verapamil Rx of COPD

Cardizem/verapamil -Blockers Digoxin Procainamide Anticoagulation

Effective

Effective

Consider follow-up EPS and RF ablation

Ineffective IV -blockers IV procainamide

Effective

FIGURE 3-105 Evaluation and management of narrow complex tachycardia. AV, Atrioventricular; COPD, chronic obstructive pulmonary disease; EPS, electrophysiologic studies; IV, intravenous; IVP, intravenous push; PSVT, paroxysmal supraventricular tachycardia; RF, radiofrequency. (From Driscoll CE et al: The family practice desk reference, ed 3, St Louis, 1996, Mosby.)

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TACHYCARDIA, WIDE COMPLEX ICD-9CM # 427.2 Paroxysmal tachycardia 427.0 Supraventricular paroxysmal tachycardia 427.42 Ventricular flutter 427.1 Ventricular paroxysmal tachycardia 427.89 Atrial tachycardia Wide complex tachycardia

Unstable DC shock

SVT with aberration vs. VT

Typical right or left bundle-branch block morphology QRS 0.12 second Normal axis

Effective

Probable VT IV procainamide IV lidocaine IV bretylium

Adenosine 6-18 mg IV push Ineffective

Not effective DC shock or overdrive pacing

Irregular rhythm

Atrial fibrillation with bundle-branch block vs. Atrial fibrillation and Wolff-Parkinson-White (WPW) syndrome

Typical right or left bundle-branch block morphology QRS 0.12 second Normal axis

Probable atrial fibrillation and WPW

Probable atrial fibrillation with bundle-branch aberration

IV procainamide or DC shock

IV procainamide cautious use of: Verapamil Cardizem -Blockers Digoxin

COMMENTS:

When in doubt of the diagnosis of a wide complex tachycardia, treat as though it is VT. Intravenous procainamide is a good initial choice, since it is effective for both SVTs and VTs. Almost all patients with wide complex tachycardia require follow-up EP testing for long-term management.

FIGURE 3-106 Evaluation and management of wide complex tachycardia. AV, Atrioventricular; EP, electrophysiologic; IV, intravenous; SVT, supraventricular tachycardia; VT, ventricular tachycardia. (From Driscoll CE et al: The family practice desk reference, ed 3, St Louis, 1996, Mosby.)

CLINICAL ALGORITHMS

Atypical QRS morphology QRS  0.12 second Bizarre axis Rate 250 beats/min

Section III

Probable atrial flutter or atrial tachycardia

IV procainamide Cautious use of: Verapamil Cardizem -Blockers Digoxin

Regular rhythm

Atypical QRS morphology QRS  0.12 second Bizarre axis AV dissociation

Probable SVT Probable AV node or AV reentr y

Stable

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TESTICULAR MASS ICD-9CM # 186.9 Testicular neoplasm M906/3 (seminoma) M9101/3 (embryonal carcinoma or teratoma) M9100/3 (choriocarcinoma) Testicular mass

Ultrasonography

Suspicious

Benign

Obtain LDH, hCG, AFP

Observe

Inguinal orchiectomy

Risk assessment

NSGCT

Seminoma

Pathology: subtype, vascular and lymphatic invasion, local invasion

Pathology: subtype (spermatocytic vs. other), local invasion

Benign

Chest CT Abdominopelvic CT Repeat serum markers

Chest, abdominopelvic CT negative Markers negative

CT negative and markers positive

CT positive

Clinical stage I

Clinical stage IS

Clinical stage II

FIGURE 3-107 Diagnosis, staging, and risk assessment of patients with testicular germ cell tumor. AFP, Alpha-fetoprotein; CT, computed tomography; hCG, human chorionic gonadotropin; LDH, lactic dehydrogenase; NSGCT, nonseminoma germ cell tumor. (From Abeloff MD: Clinical oncology, ed 2, New York, 2000, Churchill Livingstone.)

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THROMBOCYTOPENIA ICD-9CM # 287.3 Congenital or primary 287.4 Secondary 287.5 Thrombocytopenia NOS Platelet count on heparinized blood specimen

Rule out clumped platelets

Rule out drug-induced

Stop offending agent and repeat platelet count

Positive antiplatelet Ab

Diagnostic

Thrombocytopenia

Isolated thrombocytopenia

Ancillary labs Normal Coombs’ positive hemolytic anemia

Rule out ITP

Elevated LDH, schistocytes on peripheral smear

Rule out TTP

Elevated PTT, increased D-dimer, low fibrinogen

Rule out DIC

Bone marrow exam

Rule out Evans syndrome

Associated with anemia Bone marrow exam

BOX 3-7

Thrombocytopenia

Diagnostic imaging Best test None Ancillary test CT of abdomen if splenomegaly is present

Lab evaluation Best test Bone marrow exam Ancillary tests CBC, PT, PTT LDH HIV, ANA Antiplatelet Ab D-dimer Coombs’ tests

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. ANA, Antibody to nuclear antigens; CBC, complete blood count; CT, computed tomography; HIV, human immunodeficiency virus; LDH, lactic dehydrogenase; PT, prothrombin time; PTT, partial thromboplastin time.

CLINICAL ALGORITHMS

FIGURE 3-108 Evaluation of thrombocytopenia. DIC, Disseminated intravascular coagulation; ITP, idiopathic thrombocytopenic purpura; LDH, lactic dehydrogenase; PPT, partial thromboplastin time; TTP, thrombotic thrombocytopenic purpura. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

Section III

Nonhemolytic anemia

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THROMBOCYTOSIS ICD-9CM # 289.9 Increased platelets

CBC

No anemia

Decreased Hb/Hct

Bone marrow aspiration and biopsy (rule out P-Vera, CML)

Normal

CT of chest and abdomen to rule out neoplasm

Ferritin, reticulocyte count, TIBC, serum iron

Serum Fe (decreased) TIBC (increased) Ferritin level (decreased)

Iron deficiency anemia

FIGURE 3-109 Thrombocytosis diagnosis. CBC, Complete blood count; CML, chronic myelogenous leukemia; CT, computed tomography; Fe, iron; Hb/Hct, hemoglobin/hematocrit; TIBC, total iron-binding capacity. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

BOX 3-8

Thrombocytosis

Diagnostic imaging Best test None Ancillary test CT of chest and abdomen

Lab evaluation Best test Bone marrow exam Ancillary tests CBC Reticulocyte count Stool for OB 3 Serum ferritin, TIBC, iron

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. CBC, Complete blood count; CT, computed tomography; OB, occult blood; TIBC, total ironbinding capacity.

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THYROID NODULE ICD-9CM # 241.0 Nodule, thyroid Solitary thyroid nodule

Fine-needle aspiration biopsy (FNAB)

Benign (60% of samples)

Indeterminate (20% of samples)

Inadequate for diagnosis (10% of samples)

T4 suppression with L-thyroxine therapy or observation

Obtain baseline serum thyroglobulin and refer for surger y

Repeat FNAB

Benign

Malignant (10% of samples)

Indeterminate

Malignant

1. Thyroid radionuclide scan 2. Evaluate risk for malignancy

Warm nodule, high risk

Hot nodule, low risk

Surgery

Surger y

Close observation (follow every 3 to 6 months with thyroid function studies and physical examination of the thyroid)

FIGURE 3-110 Diagnostic evaluation of solitary thyroid nodule. High risk for malignancy: nodule 2 cm, age 40 yr, male sex, regional lymphadenopathy, fixation to adjacent tissues, history of prior head and neck irradiation. (From Ferri F: Practical guide to the care of the medical patient, ed 6, St Louis, 2004, Mosby.)

CLINICAL ALGORITHMS

Surgery if nodule increases in size Trial of suppression therapy if thyroid function studies are high

Section III

Cold nodule

→

Secondary hypothyroidism (hypopituitarism)

Silent thyroiditis

Graves’ disease or toxic nodule

RAIU scan

Ingestion of liothyronine (Cytomel)

→

Thyroid gland tender

Subacute thyroiditis: RAIU scan Serum thyroglobulin level

Thyrotoxicosis factitia: RAIU scan Serum thyroglobulin level

Free T4 TSH

Thyroid gland not palpable

→→

→

FIGURE 3-111 Diagnostic approach to thyroid testing. N, Normal; RAIU, radioactive iodine uptake; TRH, thyrotropin-releasing hormone; TSH, thyroid-stimulating hormone. (From Ferri FF: Practical guide to the care of the medical patient, ed 6, St Louis, 2004, Mosby.)

→

RAIU scan

T3

T3 thyrotoxicosis

Patient clinically hyperthyroid

Hyperthyroidism

Thyroid gland enlarged

RAIU scan

Normal, no additional studies necessary

Clinical signs and symptons

ICD-9CM # V77.0

RAIU scan

N free T4 N TSH

Free T4 N/ TSH

Hyperthyroidism in patient with severe illness ( T4, T3)

Patient clinically euthyroid

TRH test or serial monitoring of TSH and free T4

TSH suppression in patients receiving dopamine or corticosteroids

Hashimoto’s thyroiditis or postpartum thyroid dysfunction

Transient elevation in TSH in euthyroid patient recovering from severe illness

N/ free T4 TSH

Early hypothyroidism

Patient clinically hypothyroid

Primary hypothyroidism (thyroid gland failure)

Free T4 TSH

→

→ →

→

→ →

Serum free T4 or free thyroxine index plus Sensitive TSH

→→ →

→ →

→

→

396

THYROID TESTING

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Thyroid Testing

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TINNITUS ICD-9CM # 388.30 Tinnitus

Detailed neuro exam

Auscultation of carotids

Tympanometry

Audiometry

Normal results

Suspected AV aneurysm or stenosis of intracranial vessel

Hearing loss and/or neuro defects on exam

Carotid bruit

Rule out secondary to neuro trauma; rule out anxiety disorder with somatization; rule out psychosis

MRA of brain

MRI of brain and auditory canal

Ultrasound of carotids

Rule out MS, CVA, neoplasm

BOX 3-9

Section III

FIGURE 3-112 Tinnitus evaluation. AV, Atrioventricular; CVA, cerebrovascular accident; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; MS, multiple sclerosis. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

Tinnitus Lab evaluation Best test None Ancillary tests CBC Lipid panel

From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. CBC, Complete blood count; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging.

CLINICAL ALGORITHMS

Diagnostic imaging Best test None Ancillary tests Carotid Doppler ultrasound MRI of brain and auditory canals Brain MRA

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TRANSIENT ISCHEMIC ATTACK ICD-9CM # 435.9 Unspecified transient cerebral ischemia TIA

Head CT (R/O SDH/Mass)

ASA

Neuroimaging (MRI, vascular studies [MRA, CTA, carotid duplex]) ECG and telemetry, echocardiography

Carotid stenosis

Cardioembolic

Lacunar

Hypercoagulable (Investigate)

Consider CEA/stent

Afib

Consider AC

Thrombus

Low EF

AC

Consider AC

PFO

Continue AP Risk factor modification BP control

Consider AC

AP PFO closure (?)

FIGURE 3-113 Transient ischemic attack. AC, Anticoagulation; AP, antiplatelet; ASA, aspirin; BP, blood pressure; CEA, carotid endarterectomy; EF, ejection fraction; PFO, patent foramen ovale.

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UNCONSCIOUS PATIENT ICD-9CM # 780.09 T 0

Unconscious patient

Air way Breathing Circulation Oxygenation Start IV Fingerstick for glucose level, pulse oximetry Draw blood for 1. Glucose 2. ABG, pH 3. Na+, K+, Ca++, PO4 4. Toxicology screen 5. BUN, creatinine 6. ALT, AST, NH 3

T 5 minutes

Administer: 1. Oxygen 2. D50, 1 ampule IV, thiamine 100 mg IV 3. Naloxone 0.4-2 mg IV (flumazenil* 0.2 mg IV) T 10 minutes Examine patient

Focal or localizing signs External evidence of trauma

Signs of increased ICP

No localizing signs

T 30 minutes

Hyperventilate to PCO2 25-30 mm Hg Mannitol 0.5-1.0 g/kg IV Lasix 20 mg IV

Await metabolic workup

FIGURE 3-114 Approach to the unconscious patient. ABG, Arterial blood gas; ALT, alanine aminotransferase; AST, aspartate aminotransferase; BUN, blood urea nitrogen; CT, computed tomography; ICP, intracranial pressure. (Modified from Johnson RT, Griffin JW: Current therapy in neurologic disease, ed 5, St Louis, 1997, Mosby.)

CLINICAL ALGORITHMS

* Use of flumazenil should not be considered routine because it can precipitate seizures in certain subsets of patients.

Section III

Emergent head CT

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URETERAL CALCULI ICD-9CM # 592.9 Urinary calculus Stone size

5 mm

10 mm

5-10 mm

Observation Proximal

Success

Failure

Distal

Distal

Ureteroscopy

SWL

Ureteroscopy

SWL

SWL Ureteroscopy Success SWL

Success

Failure

Success

Ureteroscopy

Ureteroscopy ? PNL Failure

Ureteroscopy ? PNL

Failure

Proximal

SWL

Ureteroscopy

PNL

FIGURE 3-115 Management of ureteral calculi. PNL, Percutaneous nephrostolithotomy; SWL, shock wave lithotripsy. (From Noble J: Primary care medicine, ed 3, St Louis, 2001, Mosby.)

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URINARY TRACT INFECTION ICD-9CM # 595.0 595.3 595.2 590.1 590.0 590.8

Acute cystitis Trigonitis Chronic cystitis Acute pyelonephritis Chronic pyelonephritis Nonspecific pyelonephritis

Lower tract symptoms with leukocytes present

Upper tract symptoms

Asymptomatic bacteriuria*

Urine culture Confirm diagnosis with additional culture

Short course or 7-10 days of therapy

2 weeks of therapy†

Short course or 7 days of therapy† Clinical relapse

Clinical cure

Urine culture

Negative “cure”

Positive

No clinical response

Clinical response and relapse

Urine culture

Urine culture

If positive, treat for resistant organism

Reinfection*

If infrequent reinfection, treat each episode*

Relapse

Re-treat for 2 weeks*

Relapse*,‡

Imaging study

No abnormality amenable to surgery

Consider therapy for 4-6 weeks*

If further relapse, consider therapy for prolonged period*

* Consider no therapy in patients without obstructive uropathy or symptoms of urinary tract infection. † Consider imaging studies in all males with correction of significant lesions. ‡ Evaluate men for chronic bacterial prostatitis. § Consider imaging studies after three to four reinfections in women. FIGURE 3-116 Approach to the management of urinary tract infection. (Adapted from Mandell GL: Mandell, Douglas, and Bennett’s principles and practice of infectious diseases, ed 5, New York, 2000, Churchill Livingstone.)

CLINICAL ALGORITHMS

If frequent reinfection, consider long-term chemoprophylaxis*,§

Negative “cure”

Section III

Follow-up culture

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URINARY TRACT OBSTRUCTION ICD-9CM # 599.6 Acute onset

Pain (renal colic)

Acute renal failure

KUB-IVP

Ultrasound

No stone or collecting system dilatation

Stone or collecting system dilatation

No collecting system dilatation

Collecting system dilatation

Consider other causes as the etiology of pain

Appropriate intervention

Exclude false–negative results due to dehydration or too recent onset of obstruction

CT scan without contrast

Repeat ultrasound

Cause of obstruction shown

Appropriate intervention FIGURE 3-117 Scheme of a diagnostic approach to urinary tract obstruction. CT, Computed tomography; IVP, intravenous pyelography; KUB, kidney, ureter, bladder (a flat film of the abdomen without contrast medium). (From Goldman L, Ausiello D [eds]: Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.)

BOX 3-10

Diagnostic Tests Used in Obstructive Uropathy

Upper Urinary Tract Obstruction Sonography (ultrasound) Plain films of the abdomen (KUB) Excretory or intravenous pyelography Retrograde pyelography Isotopic renography Computed tomography Magnetic resonance imaging Pressure flow studies (the Whitaker test) Lower Urinary Tract Obstruction Some of the tests listed above Cystoscopy Voiding cystourethrogram Retrograde urethrography Urodynamic tests Debimetry Cystometrography Electromyography Urethral pressure profile From Klahr S: Obstructive uropathy. In Jacobson HR, Striker GE, Klahr S (eds). The principles and practice of nephrology, Toronto, 1991, BC Decker, pp 432-441. Reproduced by permission of Mosby-Year Book. KUP, Kidneys, ureter, bladder.

Tests normal

Tests abnormal

Examine stool for ova and parasites Parasitic infection

Perform biopsy

Urticaria-like dermatoses

Psychological stress Occult malignancy Physical urticaria Foods Idiopathic Biopsy nondiagnostic

Acute urticaria

Episode duration 6 weeks

Biopsy diagnostic

Systemic lupus erythematosus (SLE) Subacute cutaneous lupus erythematosus Sjögren's syndrome Mixed connective tissue disease Autoimmune thyroid disease

Parasites present

Perform history and physical exam

CLINICAL ALGORITHMS

Dermatographism Pressure urticaria Cold urticaria Solar urticaria Cholingergic urticaria Heat urticaria Aquagenic urticaria

Angiodema Dermal erythema multiforme Vasculitis Bullous pemphigoid Gyrate erythemas Urticarial vasculitis Urticaria pigmentosa/ systemic mastocytosis

Dental caries Atopic diathesis Drug sensitivity Contact urticaria Transfusion reactions Serum sickness Acute hepatitis B Idiopathic Upper respiratory infection/sinusitis Mononucleosis Food: Nuts Shellfish

FIGURE 3-118 Evaluation of urticaria. ANA, Antibody to nuclear antigens; ENA, extractable nuclear antigens; RF, rheumatoid factor. (From Healy PM, Jacobson EJ: Common medical diagnoses, ed 3, Philadelphia, 2000, WB Saunders.)

Check ANA, ENA RF thyroid antibodies

No ova and parasites

Chronic urticaria

Episode duration 6 weeks

Urticaria

ICD-9CM # 708.8 Other unspecified urticaria

Urticaria

Section III

URTICARIA

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VAGINAL PROLAPSE ICD-9CM # 618.0 Vaginal prolapse

Symptomatic

Asymptomatic

Estrogen Pelvic muscle exercises Avoid heavy lifting, straining, and girdles Weight loss Pessar y

Uterovaginal

Cystocele

Surger y

Apical

Lateral

Pessar y

Central

Anterior colporrhaphy

Paravaginal repair

Uterosacral

Uterosacral

Uterosacral ligament plication/shortening with attachment to anterior vaginal cuff

Vaginal vault suspension

Uterus only

Hysterectomy

Surgery*

Pessary

Uterus and vagina

Vagina only

Rectocele

Surger y

High-fiber diet Fiber supplement Suppository/enema

Vaginal suspension or colpectomy/colpocleisis

Uterine suspension or hysterectomy and vaginal suspension or hysterectomy and colpectomy/colpocleisis or LeFort procedure

*All procedures should include culdoplasty.

FIGURE 3-119 Management of vaginal prolapse. (From Zuspan FP [ed]: Handbook of obstetrics, gynecology, and primary care, St Louis, 1998, Mosby.)

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VERTIGO ICD-9CM # 780.4 Vertigo

Ancillary lab tests

Normal exam

Sensorineural hearing loss

Brainstem auditory sensory evoked response

Observe in otherwise healthy patient

Detailed neurologic exam (including Nylen-Bárány maneuver)

Positive Nylen-Bárány maneuver

Focal neuro deficits

Diagnostic abnormal labs

Benign positional vertigo

MRI of brain to rule out MS, neoplasm

Investigate

MRA of posterior circulation in elderly patient with risk factors

Abnormal

MRI or CT of cerebellopontine angle to rule out acoustic neuroma

FIGURE 3-120 Vertigo evaluation. CT, Computed tomography; MRA, magnetic resonance arteriography; MRI, magnetic resonance imaging; MS, multiple sclerosis. (From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby.)

BOX 3-11

Vertigo

Diagnostic imaging Best test MRI of brain Ancillary tests MRA of posterior circulation CT of cerebellopontine region if MRI is contraindicated Lab evaluation Best test None Ancillary tests CBC with differential Serum glucose, creatinine, ALT, electrolytes From Ferri FF: Ferri’s best test: a practical guide to clinical laboratory medicine and diagnostic imaging, Philadelphia, 2004, Elsevier Mosby. Alt, Alanine aminotransferase; CBC, complete blood count; CT, computed tomography; MRA, magnetic resonance arteriography; MRI, magnetic resonance imaging.

CLINICAL ALGORITHMS

Rule out Meniere’s disease, labyrinthitis

Section III

Normal

Positive

Follow

Negative

Radiation to pelvic and groin nodes

2 nodes, extranodal disease

Radiation or contralateral node dissection if only unilateral performed previously

Groin radiation

Single node, no extranodal disease

Close follow-up

Follow only

Negative

Nodal pathology

Positive

Radical vulvectomy and bilateral inguinalfemoral node dissection and pelvic exenteration

Repeat excision or perianal radiation

Margins

Radical local excision and bilateral inguinalfemoral node dissection

Midline (approaching the urethra, vagina, or anus)

Chemotherapy, radiation, and subsequent excision of tumor bed

Radiation to groin and pelvic nodes and positive margins

Modified radical vulvectomy and bilateral inguinalfemoral node dissection

Bilateral inguinal-femoral node dissection, then chemotherapy, radiation, and subsequent excision of tumor node

FIGURE 3-121 Treatment algorithm for management of patients with vulvar cancer. (From Copeland LJ: Textbook of gynecology, ed 2, Philadelphia, 2000, WB Saunders.)

Medial (involves urethra, vagina, or anus)

Chemotherapy, radiation, and possible later excision of tumor bed vs. clinical trials

Lesion location

Lateral

Positive

Negative

Pelvic nodes

Pelvic computerized tomography

Lesion location

Radical local excision and ipsilateral inguinal-femoral node dissection

Lateral

Clinically positive

ICD-9CM # 184.4 Vulvar neoplasm

Clinically negative

Groin nodes

406

VULVAR CANCER

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Vulvar Cancer

History negative

Check history

Check chemistry panel Normal

Abnormal

Normal

Recent

Gradual (lifelong)

Check TSH Normal

Abnormal

Check DHA-sulfate Normal

Abnormal

Negative

Positive

Surreptitious eating

Increased androgenic hormones

Smoking cessation Psychiatric disorder

Antipsychotics Tricyclics Estrogen Valproic acid Antihypertensives Adrenal/anabolic steroids

Prader-Willi syndrome Fröhlich’s syndrome Laurence-Moon-Biedl syndrome

Hypothyroidism

Check drug history

Genetic

Diabetes mellitus Cushing’s disease/syndrome

Check onset

CLINICAL ALGORITHMS

FIGURE 3-122 Weight gain. DHA, Dehydroepiandrosterone; TSH, thyroid-stimulating hormone. (From Healey PM: Common medical diagnosis: an algorithmic approach, ed 3, Philadelphia, 2000, WB Saunders.)

Weight gain

History positive

Check calorie intake

Increased

Dietary weight gain

ICD-9CM # 783.1 Abnormal weight gain 278.00 Obesity

Weight Gain

Section III

WEIGHT GAIN

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WEIGHT LOSS, INVOLUNTARY ICD-9CM # 783.21 Documented involuntary weight loss (IWL)

No recent institution of diuretic

Recent institution of diuretic

Observe Repeat weight at 1-2 months

Documented further IWL

Stable weight

Usual follow-up

Complete history and physical Review diet and appetite Update routine indicated cancer screening tests

Preexisting chronic illness known to cause IWL

Review prescription and over-the-counter medications for anorectic side effects

History or examination findings suggestive of illnesses known to cause IWL

No history or examination findings suggestive of cause

Reevaluate and optimize therapy

Eliminate or modify offending medications

Focus evaluation directed by abnormal findings

CBC, SMA 12 Urinalysis Thyroid-stimulating hormone level Chest x-ray Stool guaiacs

Abnormal findings

Normal findings

Follow up abnormal values

More detailed psychosocial histor y: Screen for depression, anxiety, eating disorders Screen for socioeconomic factors (poverty, lack of transportation, isolation) Screen for substance abuse Consider food diar y

Probable psychosocial cause detected

No abnormalities detected

Treat appropriately

Unexplained IWL

Follow at close intervals (high risk) FIGURE 3-123 Involuntary weight loss. CBC, Complete blood count. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

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Laboratory Tests and Interpretation of Results

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This section contains more than 150 commonly performed laboratory tests. In general, the tests are approached with the following format: 1. Laboratory test 2. Normal range in adult patients 3. Common abnormalities, such as positive test, increased or decreased value 4. Causes of abnormal result

The normal ranges may differ slightly, depending on the laboratory. The reader should be aware of the “normal range” of the particular laboratory performing the test. Every attempt has been made to present current laboratory test data, with emphasis on practical considerations.

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●

Acetylcholine Receptor (ACHR) Antibody

Albumin

ACETYLCHOLINE RECEPTOR (ACHR) ANTIBODY

ALANINE AMINOTRANSFERASE (ALT, SGPT)

Normal: 0.03 nmol/L Elevated in: Myasthenia gravis. Changes in AChR concentration correlate with the clinical severity of myasthenia gravis following therapy and during therapy with prednisone and immunosuppressants. False-positive AChR antibody results may be found in patients with Eaton-Lambert syndrome.

ACID-BASE REFERENCE VALUES; see Tables 4-1 and 4-2.

Normal range: 0-35 U/L Elevated in: Liver disease (hepatitis, cirrhosis), hepatic congestion, infectious mononucleosis, myocardial infarction, myocarditis, severe muscle trauma, dermatomyositis/polymyositis, muscular dystrophy, drugs (antibiotics, narcotics, antihypertensive agents, heparin, labetalol, statins, NSAIDs, amiodarone, chlorpromazine, phenytoin), malignancy, renal and pulmonary infarction, convulsions, shock liver

ACID PHOSPHATASE (serum)

ALBUMIN (serum)

Normal range: 0-5.5 U/L Elevated in: Carcinoma of prostate, other neoplasms (breast, bone), Paget’s disease, osteogenesis imperfecta, malignant invasion of bone, Gaucher’s disease, multiple myeloma, myeloproliferative disorders, benign prostatic hypertrophy, prostatic palpation or surgery, hyperparathyroidism, liver disease, chronic renal failure, idiopathic thrombocytopenic purpura, bronchitis

Normal range: 4-6 g/dl Elevated in: Dehydration (relative increase) Decreased in: Liver disease, nephrotic syndrome, poor nutritional status, rapid IV hydration, protein-losing enteropathies (inflammatory bowel disease), severe burns, neoplasia, chronic inflammatory diseases, prolonged immobilization, lymphomas, hypervitaminosis A, chronic glomerulonephritis

411

Commonly Used Acid-Base Reference Values for Arterial and Venous Plasma or Serum (Averaged from Various Sources)

TABLE 4-1

ARTERIAL Conventional Units pH Pco2 Po2 HCO3 (CO2 combining power) CO2 content

VENOUS SI Units*

7.40 (7.35-7.45) 40 mm Hg (35-45) 80-100 mm Hg

7.40 (7.35-7.45) 5.33 kPa (4.67-6.10) 10.66-13.33 kPa

24 mEq/L (20-28) 25 mEq/L (22-28)

24 mmol/L (20-28) 25 mmol/L (22-28)

Conventional Units 7.37 (7.32-7.42) 45 mm Hg (45-50) 40 mm Hg (37-43) 26 mEq/L (22-30) 27 mEq/L (24-30)

SI Units* 7.37 (7.32-7.42) 6.10 kPa (5.33-6.67) 5.33 kPa (4.93-5.73) 26 mmol/L (22-30) 27 mmol/L (24-30)

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. *International system.

Summary of Laboratory Findings in Primary Uncomplicated Respiratory and Metabolic Acid-Base Disorders*

TABLE 4-2

pH

Base Excess

Acute primary respiratory hypoactivity (respiratory acidosis) Acute primary respiratory hyperactivity (respiratory alkalosis) Uncompensated metabolic acidosis Uncompensated metabolic alkalosis Partially compensated metabolic acidosis Partially compensated metabolic alkalosis Chronic primary respiratory hypoactivity (compensated respiratory acidosis) Fully compensated metabolic alkalosis Chronic primary respiratory hyperactivity (compensated respiratory alkalosis) Fully compensated metabolic acidosis

Increase

Decrease

Normal/positive

Decrease

Increase

Normal/negative

Normal Normal Decrease Increase Increase

Decrease Increase Decrease Increase Normal

Negative Positive Negative Positive Positive

Increase Decrease

Normal Normal

Positive Negative

Decrease

Normal

Negative

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. *Base excess results refer to negative () values more than 22 and positive () values more than 2.

LABORATORY TESTS AND INTERPRETATION

PCO2

Section IV

Disorder

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Aldolase ALDOLASE (serum) Normal range: 0-6 U/L Elevated in: Muscular dystrophy, rhabdomyolysis, dermatomyositis/polymyositis, trichinosis, acute hepatitis and other liver diseases, myocardial infarction, prostatic carcinoma, hemorrhagic pancreatitis, gangrene, delirium tremens, burns Decreased in: Loss of muscle mass, late stages of muscular dystrophy

ALDOSTERONE Normal range: Recumbent: 50-150 ng/L Upright: 150-300 ng/L Elevated in: Primary aldosteronism, secondary aldosteronism, pseudoprimary aldosteronism Decreased in: Patient with hypertension: diabetes mellitus, Turner’s syndrome, acute alcohol intoxication, excess secretion of deoxycorticosterone, corticosterone, and 18-hydroxycorticosterone Patient without hypertension: Addison’s disease, hypoaldosteronism resulting from renin deficiency, isolated aldosterone deficiency

ALKALINE PHOSPHATASE (serum) Normal range: 30-120 U/L Elevated in: LIVER AND BILIARY TRACT ORIGIN Extrahepatic bile duct obstruction Intrahepatic biliary obstruction Liver cell acute injury Liver passive congestion Drug-induced liver cell dysfunction Space-occupying lesions Primary biliary cirrhosis Sepsis BONE ORIGIN (OSTEOBLAST HYPERACTIVITY) Metastatic tumor with osteoblastic reaction Fracture healing Paget’s disease of bone CAPILLARY ENDOTHELIAL ORIGIN Granulation tissue formation (active) OTHER Thyrotoxicosis Benign transient hyperphosphatasemia Primary hyperparathyroidism Decreased in: Hypothyroidism, pernicious anemia, hypophosphatemia, hypervitaminosis D, malnutrition

ALT; see ALANINE AMINOTRANSFERASE AMMONIA (serum) Normal range: 10-80 g/dl Elevated in: Hepatic failure, hepatic encephalopathy, portacaval shunt, drugs (diuretics, polymyxin B, methicillin) Decreased in: Drugs (neomycin, lactulose, tetracycline), renal failure

AMYLASE (serum) Normal range: 0-130 U/L Elevated in: Acute pancreatitis, pancreatic neoplasm, abscess, pseudocyst, ascites, macroamylasemia, perforated peptic ulcer, intestinal obstruction, intestinal infarction, acute cholecystitis, appendicitis, salivary gland inflammation, peritonitis, burns, diabetic ketoacidosis, renal insufficiency, drugs (morphine), carcinomatosis (of lung, esophagus, ovary), acute ethanol ingestion,

●

Anti-Smooth Muscle Antibody mumps, prostate tumors, post–endoscopic retrograde cholangiopancreatography Decreased in: Advanced chronic pancreatitis, hepatic necrosis, cystic fibrosis

ANA; see ANTINUCLEAR ANTIBODY ANCA; see ANTINEUTROPHIL CYTOPLASMIC ANTIBODY ANION GAP Normal range: 9-14 mEq/L Elevated in: Lactic acidosis, ketoacidosis (diabetes, alcoholic starvation), uremia (chronic renal failure), ingestion of toxins (paraldehyde, methanol, salicylates, ethylene glycol), hyperosmolar nonketotic coma, antibiotics (carbenicillin) Decreased in: Hypoalbuminemia, severe hypermagnesemia, IgG myeloma, lithium toxicity, laboratory error (falsely decreased sodium or overestimation of bicarbonate or chloride), hypercalcemia of parathyroid origin, antibiotics (e.g., polymyxin)

ANTICARDIOLIPIN ANTIBODY (ACA) Normal range: Negative: Test includes detection of IgG, IgM, and IgA antibody to phospholipid, cardiolipin Present in: Antiphospholipid antibody syndrome, chronic hepatitis C

ANTICOAGULANT; see CIRCULATING ANTICOAGULANT ANTI-DNA Normal range: Absent Present in: Systemic lupus erythematosus, chronic active hepatitis, infectious mononucleosis, biliary cirrhosis

ANTIMITOCHONDRIAL ANTIBODY Normal range: 1:20 titer Elevated in: Primary biliary cirrhosis (85% to 95%), chronic active hepatitis (25% to 30%), cryptogenic cirrhosis (25% to 30%)

ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA) Positive test: Cytoplasmic pattern (cANCA): positive in Wegener’s granulomatosis Perinuclear pattern (pANCA): positive in inflammatory bowel disease, primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune chronic active hepatitis, crescenteric glomerulonephritis

ANTINUCLEAR ANTIBODY (ANA) Normal range: 1:20 titer Positive test: Systemic lupus erythematosus (more significant if titer 1:160), drugs (phenytoin, ethosuximide, primidone, methyldopa, hydralazine, carbamazepine, penicillin, procainamide, chlorpromazine, griseofulvin, thiazides), chronic active hepatitis, age over 60 years (particularly age over 80 years), rheumatoid arthritis, scleroderma, mixed connective tissue disease, necrotizing vasculitis, Sjögren’s syndrome, tuberculosis, pulmonary interstitial fibrosis. Table 4-3 describes diseases associated with ANA subtypes. Fig. 4-1 illustrates various fluorescent ANA test patterns.

ANTI-RNP ANTIBODY; see EXTRACTABLE NUCLEAR ANTIGEN ANTI-SM (ANTI-SMITH) ANTIBODY; see EXTRACTABLE NUCLEAR ANTIGEN ANTI-SMOOTH MUSCLE ANTIBODY; see SMOOTH MUSCLE ANTIBODY

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●

Antinuclear Antibody A

B

E

Antinuclear Antibody

C

F

413

D

G

FIGURE 4-1 Fluorescent antinuclear antibody test patterns (HEP-2 cells). A, Solid (homogeneous). B, Peripheral (rim). C, Speckled. D, Nucleolar. E, Anticentromere. F, Antimitochondrial. G, Normal (nonreactive). (From Ravel R [ed]: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

TABLE 4-3

Disease-Associated ANA Subtypes

Nuclear Location

Disease(s)

“Native” DNA (dsDNA, or dsDNA/ssDNA complex) sNP

SLE (60%-70%; range, 35%-75%) —also PSS (5%-55%), MCTD (11%-25%), RA (5%-40%), DM (5%-25%), SS (5%) SLE (50%) —also other collagen diseases SLE (52%) —also MCTD (8%), RA (3%) Drug-induced SLE (95%) —also SLE (30%), RA (15%-24%)

DNP (DNA-histone complex) Histones ENA Sm RNP (U1-RNP) SS-A (Ro)* SS-B (La) Scl-70* Centromere* Nucleolar RAP (RANA)

ssDNA

Disease(s)

Mitochondrial

Primary biliary cirrhosis (90%-100%) —also CAH (7%-30%), cryptogenic cirrhosis (30%), acute hepatitis, viral hepatitis (3%), other liver diseases (0%-20%), SLE (5%), SS and PSS (8%) Chronic active hepatitis (60%-80%), Hashimoto’s thyroiditis (97%) SLE (5%-12%) Chronic active hepatitis (60%-91%) —also cryptogenic cirrhosis (28%), acute hepatitis, viral hepatitis (5%-87%), infectious mononucleosis (81%), MS (40%-50%), malignancy (67%), PBC (10%-50%)

Microsomal† Ribosomal Smooth muscle‡

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. CAH, Chronic active hepatitis; DM, dermatomyositis; GN, glomerulonephritis; MS, multiple sclerosis; PBC, primary biliary cirrhosis; SS, Sjögren’s syndrome. *Not detected using rat or mouse liver or kidney tissue method. †Not detected by cultured cell method. ‡Detected by cultured cells but better with rat or mouse tissue.

LABORATORY TESTS AND INTERPRETATION

Cytoplasmic Location

Section IV

Jo-1 PM-1

SLE (30%-40%; range, 28%-40%) —also MCTD (0%-8%) MCTD (in high titer without any other ANA subtype present: 95%-100%) —also SLE (26%-50%), PSS (11%-22%), RA (10%), SS (3%) SS without RA (60%-70%) —also SLE (26%-50%), neonatal SLE (over 95%), PSS (30%), MCTD (50%), SS with RA (9%), PBC (15%-19%) SS without RA (40%-60%) —also SLE (5%-15%), SS with RA (5%) PSS (15%-43%) CREST syndrome (70%-90%; range 57%-96%) —also PSS (4%-20%), PBC (12%) PSS (scleroderma) (54%-90%) —also SLE (25%-26%), RA (9%) SS with RA (60%-76%) —also SS without RA (5%) Polymyositis (30%) Polymyositis or PMS/PSS overlap syndrome (60%-90%) —also DM (17%) SLE (60%-70%) —also CAH, infectious mononucleosis, RA, chronic GN, chronic infections, PBC

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Antistreptolysin O Titer ANTISTREPTOLYSIN O TITER (Streptozyme, ASLO titer) Normal range for adults: 160 Todd units Elevated in: Streptococcal upper airway infection, acute rheumatic fever, acute glomerulonephritis, increased levels of -lipoprotein NOTE: A fourfold increase in titer between acute and convalescent specimens is diagnostic of streptococcal upper airway infection regardless of the initial titer.

ARTERIAL BLOOD GASES Normal range: Po2: 75-100 mm Hg Pco2: 35-45 mm Hg HCO3: 24-28 mEq/L pH: 7.35-7.45 Abnormal values: Acid-base disturbances (see the following) METABOLIC ACIDOSIS Metabolic acidosis with increased AG (AG acidosis)

Lactic acidosis Ketoacidosis (diabetes mellitus, alcoholic ketoacidosis) Uremia (chronic renal failure) Ingestion of toxins (paraldehyde, methanol, salicylate, ethylene glycol) High-fat diet (mild acidosis) Metabolic acidosis with normal AG (hyperchloremic acidosis)

Renal tubular acidosis (including acidosis of aldosterone deficiency) Intestinal loss of HCO3 (diarrhea, pancreatic fistula) Carbonic anhydrase inhibitors (e.g., acetazolamide) Dilutional acidosis (as a result of rapid infusion of bicarbonatefree isotonic saline) Ingestion of exogenous acids (ammonium chloride, methionine, cystine, calcium chloride) Ileostomy Ureterosigmoidostomy Drugs: amiloride, triamterene, spironolactone, -blockers RESPIRATORY ACIDOSIS Pulmonary disease (COPD, severe pneumonia, pulmonary edema, interstitial fibrosis) Airway obstruction (foreign body, severe bronchospasm, laryngospasm) Thoracic cage disorders (pneumothorax, flail chest, kyphoscoliosis) Defects in muscles of respiration (myasthenia gravis, hypokalemia, muscular dystrophy) Defects in peripheral nervous system (amyotrophic lateral sclerosis, poliomyelitis, Guillain-Barré syndrome, botulism, tetanus, organophosphate poisoning, spinal cord injury) Depression of respiratory center (anesthesia, narcotics, sedatives, vertebral artery embolism or thrombosis, increased intracranial pressure) Failure of mechanical ventilator METABOLIC ALKALOSIS It is divided into chloride-responsive (urinary chloride 15 mEq/L) and chloride-resistant forms (urinary chloride level 15 mEq/L) Chloride-responsive

Vomiting Nasogastric (NG) suction Diuretics Posthypercapnic alkalosis Stool losses (laxative abuse, cystic fibrosis, villous adenoma) Massive blood transfusion Exogenous alkali administration Chloride-resistant

Hyperadrenocorticoid states (Cushing’s syndrome, primary hyperaldosteronism, secondary mineralocorticoidism [licorice, chewing tobacco])

●

Aspartate Aminotransferase Hypomagnesemia Hypokalemia Bartter’s syndrome RESPIRATORY ALKALOSIS Hypoxemia (pneumonia, pulmonary embolism, atelectasis, highaltitude living) Drugs (salicylates, xanthines, progesterone, epinephrine, thyroxine, nicotine) Central nervous system (CNS) disorders (tumor, cerebrovascular accident [CVA], trauma, infections) Psychogenic hyperventilation (anxiety) Hepatic encephalopathy Gram-negative sepsis Hyponatremia Sudden recovery from metabolic acidosis Assisted ventilation

ARTHROCENTESIS FLUID Interpretation of results: 1. Color: Normally it is clear or pale yellow; cloudiness indicates inflammatory process or presence of crystals, cell debris, fibrin, or triglycerides. 2. Viscosity: Normally it has a high viscosity because of hyaluronate; when fluid is placed on a slide, it can be stretched to a string 2 cm in length before separating (low viscosity indicates breakdown of hyaluronate [lysosomal enzymes from leukocytes] or the presence of edema fluid). 3. Mucin clot: Add 1 ml of fluid to 5 ml of a 5% acetic acid solution and allow 1 minute for the clot to form; a firm clot (does not fragment on shaking) is normal and indicates the presence of large molecules of hyaluronic acid (this test is nonspecific and infrequently done). 4. Glucose: Normally it approximately equals serum glucose level; a difference of more than 40 mg/dl is suggestive of infection. 5. Protein: Total protein concentration is 2.5 g/dl in the normal synovial fluid; it is elevated in inflammatory and septic arthritis. 6. Microscopic examination for crystals: a. Gout: Monosodium urate crystals b. Pseudogout: Calcium pyrophosphate dihydrate crystals

ASLO TITER: see ANTISTREPTOLYSIN O TITER ASPARTATE AMINOTRANSFERASE (AST, SGOT) Normal range: 0-35 U/L Elevated in: HEART Acute myocardial infarction Pericarditis (active: some cases) LIVER Hepatitis virus, Epstein-Barr, or cytomegalovirus infection Active cirrhosis Liver passive congestion or hypoxia Alcohol or drug-induced liver dysfunction Space-occupying lesions (active) Fatty liver Extrahepatic biliary obstruction (early) Drug-induced SKELETAL MUSCLE Acute skeletal muscle injury Muscle inflammation (infectious or noninfectious) Muscular dystrophy (active) Recent surgery Delirium tremens

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B-Type Natriuretic Peptide KIDNEY Acute injury or damage Renal infarct OTHER Intestinal infarction Shock Cholecystitis Acute pancreatitis Hypothyroidism Heparin therapy (60%-80% of cases)

B-TYPE NATRIURETIC PEPTIDE Normal range: Up to 100 pg/mL Elevated in: Heart failure. This test is useful in the emergency department setting to differentiate heart failure patients from those with chronic obstructive pulmonary disease presenting with dyspnea.

BILIRUBIN, DIRECT (conjugated bilirubin) Normal range: 0-0.2 mg/dl Elevated in: Hepatocellular disease, biliary obstruction, druginduced cholestasis, hereditary disorders (Dubin-Johnson syndrome, Rotor’s syndrome)

BILIRUBIN, INDIRECT (unconjugated bilirubin)

BILIRUBIN, TOTAL

BLEEDING TIME (modified Ivy method) Normal range: 2 to 91⁄2 min Elevated in: Thrombocytopenia, capillary wall abnormalities, platelet abnormalities (Bernard-Soulier disease, Glanzmann’s disease), drugs (aspirin, warfarin, antiinflammatory medications, streptokinase, urokinase, dextran, -lactam antibiotics, moxalactam), disseminated intravascular coagulation, cirrhosis, uremia, myeloproliferative disorders, von Willebrand’s disease

415

BUN; see UREA NITROGEN C3; see COMPLEMENT C3 C4; see COMPLEMENT C4 CALCIUM (serum) Normal range: 8.8-10.3 mg/dl ELEVATED RELATIVELY COMMON Neoplasia (noncutaneous) Bone primary Myeloma Acute leukemia Nonbone solid tumors Breast Lung Squamous nonpulmonary Kidney Neoplasm secretion of parathyroid hormone-related protein (PTHrP, “ectopic PTH”) Primary hyperparathyroidism Thiazide diuretics Tertiary (renal) hyperparathyroidism Idiopathic Spurious (artifactual) hypercalcemia Dehydration Serum protein elevation Laboratory technical problem RELATIVELY UNCOMMON Neoplasia (less common tumors) Sarcoidosis Hyperthyroidism Immobilization (mostly seen in children and adolescents) Diuretic phase of acute renal tubular necrosis Vitamin D intoxication Milk-alkali syndrome Addison’s disease Lithium therapy Idiopathic hypercalcemia of infancy Acromegaly Theophylline toxicity • Table 4-4 describes the laboratory differential diagnosis of hypercalcemia. DECREASED Artifactual Hypoalbuminemia Hemodilution Primary hypoparathyroidism Pseudohypoparathyroidism Vitamin D–related Vitamin D deficiency Malabsorption Renal failure Magnesium deficiency Sepsis Chronic alcoholism Tumor lysis syndrome Rhabdomyolysis Alkalosis (respiratory or metabolic) Acute pancreatitis Drug-induced hypocalcemia Large doses of magnesium sulfate Anticonvulsants Mithramycin Gentamicin Cimetidine • Table 4-5 describes the laboratory differential diagnosis of hypocalcemia.

LABORATORY TESTS AND INTERPRETATION

Normal range: 0-1.0 mg/dl Elevated in: Liver disease (hepatitis, cirrhosis, cholangitis, neoplasm, biliary obstruction, infectious mononucleosis), hereditary disorders (Gilbert’s disease, Dubin-Johnson syndrome), drugs (steroids, diphenylhydantoin, phenothiazines, penicillin, erythromycin, clindamycin, captopril, amphotericin B, sulfonamides, azathioprine, isoniazid, 5-aminosalicylic acid, allopurinol, methyldopa, indomethacin, halothane, procainamide, tolbutamide, labetalol), hemolysis, pulmonary embolism or infarct, hepatic congestion secondary to congestive heart failure

Calcium

Section IV

Normal range: 0-1.0 mg/dl Elevated in: A. Increased bilirubin production (if normal liver, serum unconjugated bilirubin is usually less than 4 mg/100 ml) 1. Hemolytic anemia a. Acquired b. Congenital 2. Resorption from extravascular sources a. Hematomas b. Pulmonary infarcts 3. Excessive ineffective erythropoiesis a. Congenital (congenital dyserythropoietic anemias) b. Acquired (pernicious anemia, severe lead poisoning; if present, bilirubinemia is usually mild) B. Defective hepatic unconjugated bilirubin clearance (defective uptake or conjugation) 1. Severe liver disease 2. Gilbert’s syndrome 3. Crigler-Najjar type I or II 4. Drug-induced inhibition 5. Portacaval shunt 6. Congestive heart failure 7. Hyperthyroidism (uncommon)

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Carcinoembryonic Antigen

TABLE 4-4

Cerebrospinal Fluid

Laboratory Differential Diagnosis of Hypercalcemia PLASMA TESTS

URINE TESTS

Diagnosis

Ca

PO4

PTH

25(OH)D

1,25(OH)2D

cAMP

TmP/GFR

Ca

Comments

Primary hyperparathyroidism MEN I

↑

N/↓

↑

N

N/↑

↑

↓

↑

Parathyroid adenoma most common

MEN IIa MEN IIb FHH

Malignancy Solid tumor— humoral Solid tumor— osteolytic Lymphoma Granulomatous disease Vitamin D intoxication Hyperthyroidism

↑

N

N/↑

N

N

N/↑

N/↓

↓↓

↑

N/↓

↓

N

N

↑

↓

↑↑

↑

N/↑

↓

N

N

↓

↑

↑↑

↑ ↑

N/↑ N/↑

↓ ↓

N/↓ N/↓

↑ ↑↑

↓ ↓

↑ ↑

↑↑ ↑↑

↑

N/↑

↓

↑↑

N

↓

↑

↑↑

↑

N

↓

N

N

N

N

↑↑

Parathyroid hyperplasia; also includes pituitary and pancreatic neoplasms Parathyroid hyperplasia; also includes medullary thyroid carcinoma and pheochromocytoma Parathyroid disease uncommon, primarily medullary thyroid carcinoma and pheochromocytoma Autosomal dominant inheritance; hypercalcemia present within first decade; benign Primarily epidermoid tumors; PTH-related protein(s) is mediator

Sarcoid most common etiology

Plasma concentrations of T4 and/or T3 are elevated

From Moore WT, Eastman RC: Diagnostic endocrinology, ed 2, St Louis, 1996, Mosby. Ca, Calcium; cAMP, cyclic adenosine monophosphate; FHH, familial hypocalciuric hypercalcemia; GFR, glomerular filtration rate; MEN, multiple endocrine neoplasia; 25(OH)D, 25 hydroxyvitamin D; PO4, phosphate; PTH, parathormone; T3, triiodothyronine; T4, thyroxine; TmP, renal threshold for phosphorus.

CARCINOEMBRYONIC ANTIGEN (CEA) Normal range: Nonsmokers: 0-2.5 ng/ml Smokers: 0-5 ng/ml Elevated in: Colorectal carcinomas, pancreatic carcinomas, and metastatic disease (usually produce higher elevations: 20 ng/ml) Carcinomas of the esophagus, stomach, small intestine, liver, breast, ovary, lung, and thyroid (usually produce lesser elevations) Benign conditions (smoking, inflammatory bowel disease, hypothyroidism, cirrhosis, pancreatitis, infections) (usually produce levels 10 ng/ml)

CAROTENE (serum) Normal range: 50-250 g/dl Elevated in: Carotenemia, chronic nephritis, diabetes mellitus, hypothyroidism, nephrotic syndrome, hyperlipidemia Decreased in: Fat malabsorption, steatorrhea, pancreatic insufficiency, lack of carotenoids in diet, high fever, liver disease

CEA; see CARCINOEMBRYONIC ANTIGEN CEREBROSPINAL FLUID (CSF) Interpretation of results: 1. Appearance of the fluid a. Clear: normal. b. Yellow color (xanthochromia) in the supernatant of centrifuged CSF within 1 hour or less after collection is usually the result of previous bleeding (subarachnoid hemorrhage); it may also be caused by increased CSF protein, melanin from meningeal melanosarcomas, or carotenoids.

c. Pinkish color is usually the result of a bloody tap; the color generally clears progressively from tubes 1 to 4 (the supernatant is usually crystal clear in traumatic taps). d. Turbidity usually indicates the presence of leukocytes (bleeding introduces approximately 1 WBC/500 RBCs into the CSF). 2. CSF pressure: elevated pressure can be seen with meningitis, meningoencephalitis, mass lesions, and intracerebral bleeding. 3. Cell count: in the adult the CSF is normally free of cells (although up to 5 mononuclear cells/mm3 is considered normal); the presence of granulocytes is never normal. a. Neutrophils: seen in bacterial meningitis, early viral meningoencephalitis, and early tuberculosis (TB) meningitis. b. Increased lymphocytes: TB meningitis, viral meningoencephalitis, syphilitic meningoencephalitis, fungal meningitis. 4. Protein: serum proteins are generally too large to cross the normal blood-CSF barrier; however, increased CSF protein is seen with meningeal inflammation, traumatic tap, increased CNS synthesis, tissue degeneration, obstruction to CSF circulation, and Guillain-Barré syndrome. 5. Glucose a. Decreased glucose is seen with bacterial meningitis, TB meningitis, fungal meningitis, subarachnoid hemorrhage, and some cases of viral meningitis. b. A mild increase in CSF glucose can be seen in patients with very elevated serum glucose levels. Table 4-6 describes cerebrospinal fluid findings in central nervous system disorders.

≠ ↑ N N/↓

N/↓ N/↓

↓

↓

↓ ↓

↓

↓

Hypoparathyroidism Pseudohypoparathyroidism Type I

Type II Vitamin D deficiency

Vitamin D– dependent rickets Type I

↓ ↓ ↓ ↑

↑ ↑

↓ ↓ ↓ N/↓

↓ ↑↑

N

N

N

N ↓↓

N

cAMP

1,25(OH)2D

25(OH)D

↑ ↑

NC

↑↑

cAMP AFTER PTH

↓

↓

↑ ↓

↑

↑

TmP/GFR

↑ ↓

↑

↓↓

TmP/GFR AFTER PTH

↓↓

↓↓

N/↓ ↓↓

N/↓

N/↓

Ca

Deficient activity of renal 25(OH)D-1ahydroxylase Resistance to 1,25(OH)2D

Resistance to PTH; patients may have Albright’s hereditary osteodystrophy and resistance to multiple hormones Renal resistance to cAMP Deficient supply (e.g., nutrition) or absorption (e.g., pancreatic insufficiency) of vitamin D

Deficiency of PTH

COMMENTS

● Calcium

Section IV

LABORATORY TESTS AND INTERPRETATION

From Moore WT, Eastman RC: Diagnostic endocrinology, ed 2, St Louis, 1996, Mosby. Ca, Calcium; cAMP, cyclic adenosine monophosphate; FHH, familial hypocalciuric hypercalcemia; GFR, glomerular filtration rate; MEN, multiple endocrine neoplasia; NC, no change or small increase; (OH)D, hydroxycalciferol D; PO4, phosphate; PTH, parathyroid hormone; T3, triiodothyronine; T4, thyroxine; TmP, renal threshold for phosphorus.

↑↑

↑↑

↑↑ ↑↑

↑↑

N/↓

PTH

URINE TESTS

Calcium

Type II

PO4

PLASMA TESTS

Laboratory Differential Diagnosis of Hypocalcemia

Ca

DIAGNOSIS

TABLE 4-5

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417

Usually elevated

Usually elevated

Elevated

Syphilis (acute) and leptospirosis

Amebic (Naegleria) meningoencephalitis

1000-10,000 or more; PMNs predominate

5-500; PMNs early but mononuclear cells predominate through most of the course. Cryptococcal meningitis may have no cellular inflammatory response 50-500; lymphocytes predominate

50-500

50-200

25-500

Normal or slightly decreased

Usually normal

50; decreases with time if treatment is not provided

50 in most cases; decreases with time if treatment is not provided

Generally normal; may be decreased to 40 in some viral diseases, particularly mumps (15%-20% of cases)

Positive CSF serology. Spirochetes not demonstrable by usual techniques of smear or culture; darkfield examination may be positive Mobile amebae may be seen by hanging-drop examination of CSF at room temperature

Acid-fast organisms almost never seen on smear. Organisms may be recovered in culture of large volumes of CSF. Mycobacterium tuberculosis may be detected by PCR of CSF Budding yeast may be seen. Organisms may be recovered in culture. Cryptococcal antigen (CSF and serum) may be positive in cryptococcal infection

Organisms usually seen on Gram stain and recovered by culture. Latex agglutination of CSF usually positive Organisms may be seen on Gram stain. Latex agglutination CSF may be positive. Pretreatment may render CSF sterile HSV encephalitis is suggested by focal seizures or by focal findings on CT or MRI scans or EEG. Enteroviruses and HSV infrequently recovered from CSF. HSV and enteroviruses may be detected by PCR of CSF

●

Fungal meningitis

100-3000; may be higher in presence of block

Usually 50-200

Rarely 1000 cells. Eastern equine encephalitis and lymphocytic choriomeningitis (LCM) may have cell counts of several thousand. PMNs early but mononuclear cells predominate through most of the course

Normal or slightly elevated (80-150)

Viral meningitis or meningoencephalitis

10-500; PMNs early, but lymphocytes predominate through most of the course

Usually 100-500

5-10,000; PMNs usual but mononuclear cells may predominate if pretreated for extended period of time

Normal or elevated

Partially treated bacterial meningitis

Uncommon Forms of Meningitis Tuberculous Usually elevated meningitis

Decreased, usually 40 (or 66% serum glucose)

Usually 100-500

100-10,000 or more; usually 300-2000; PMNs predominate

Comments

Cerebrospinal Fluid

Normal or decreased

50 (or 75% serum glucose)

20-45

5, 75% lymphocytes

Normal 50-80 Common Forms of Meningitis Acute bacterial Usually elevated meningitis (100-300)

Glucose (mg/dL)

Protein (mg/dL)

Pressure (mm H2O)

Leukocytes (mm3)

Condition

Cerebrospinal Fluid Findings in Central Nervous System Disorders

418

TABLE 4-6

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Cerebrospinal Fluid

Slightly elevated to very high

No organisms on smear or culture unless abscess ruptures into ventricular system No organisms on smear or culture of CSF unless meningitis also present; organisms found on tap of subdural fluid No organisms on smear or culture of CSF No organisms on smear or culture of CSF Epithelial cells may be seen within CSF by use of polarized light in some children with dermoids No specific findings No organisms on smear or culture. LE preparation may be positive. Positive neuronal and ribosomal P protein antibodies in CSF Cytology may be positive

Normal unless abscess ruptures into ventricular system Normal

Normal Normal Normal or slightly decreased

Normal Normal or slightly decreased Normal to decreased (20-40)

75-500

100-500

50-200 50-400 50-100

40-100 100

50-1000

5-200; CSF rarely acellular; lymphocytes predominate; if abscess ruptures into ventricle, PMNs predominate and cell count may reach 100,000 100-5000; PMNs predominate

10-500; lymphocytes predominate 10-100; lymphocytes predominate 100-1000 or more; PMNs predominate

0-100; mononuclear 0-500; PMNs usually predominate; lymphocytes may be present 0-100 or more; mononuclear or blast cells

Cerebrospinal Fluid ● Cerebrospinal Fluid

Section IV

LABORATORY TESTS AND INTERPRETATION

From Behrman RE: Nelson textbook of pediatrics, ed 16, Philadelphia, 2000, WB Saunders. CSF, Cerebrospinal fluid; EEG, electroencephalogram; HSV, herpes simplex virus; PCR, polymerase chain reaction; PMN, polymorphonuclear neutrophils.

Tumor, leukemia

Systemic lupus erythematosus with CNS involvement

Normal to slightly elevated Usually low, with spinal block Usually elevated

Cerebral epidural abscess Spinal epidural abscess Chemical (drugs, dermoid cysts, myelography dye) Noninfectious Causes Sarcoidosis

Normal or elevated slightly Slightly elevated

Usually elevated (100-300)

Subdural empyema

Brain and Parameningeal Abscesses Usually elevated Brain abscess (100-300)

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Ceruloplasmin CERULOPLASMIN (serum) Normal range: 20-35 mg/dl Elevated in: Estrogens, neoplastic diseases (leukemias, Hodgkin’s lymphoma, carcinomas), inflammatory states, systemic lupus erythematosus, primary biliary cirrhosis, rheumatoid arthritis Decreased in: Wilson’s disease (values often 10 mg/dl), nephrotic syndrome, advanced liver disease, malabsorption, total parenteral nutrition

CHOLESTEROL, HIGH-DENSITY LIPOPROTEIN; see HIGH-DENSITY LIPOPROTEIN CHOLESTEROL

colitis, rheumatoid arthritis, hemophilia, neoplasms, chronic inflammatory states, AIDS, nephrotic syndrome NOTE: The name is a misnomer because these patients are prone to hypercoagulability and thrombosis.

CK; see CREATINE KINASE CLOSTRIDIUM DIFFICILE TOXIN ASSAY (stool) Normal: Negative Detected in: Antibiotic-associated diarrhea and pseudomembranous colitis

COAGULATION FACTORS; see Table 4-7 for characteristics of coagulation factors

CHOLESTEROL, LOW-DENSITY LIPOPROTEIN; see LOW-DENSITY LIPOPROTEIN CHOLESTEROL CHOLESTEROL, TOTAL Normal range: Varies with age Generally 200 mg/dl Elevated in: Primary hypercholesterolemia, biliary obstruction, diabetes mellitus, nephrotic syndrome, hypothyroidism, primary biliary cirrhosis, high-cholesterol diet, myocardial infarction, drugs (steroids, phenothiazines) Decreased in: Starvation, malabsorption, sideroblastic anemia, thalassemia, abetalipoproteinemia, hyperthyroidism, Cushing’s syndrome, hepatic failure, multiple myeloma, polycythemia vera, chronic myelocytic leukemia, myeloid metaplasia, Waldenström’s macroglobulinemia, myelofibrosis

CIRCULATING ANTICOAGULANT (lupus anticoagulant) Normal: Negative Detected in: Systemic lupus erythematosus, drug-induced lupus, long-term phenothiazine therapy, multiple myeloma, ulcerative

TABLE 4-7

Complement

Factor reference ranges: V: 10% VII: 10% VIII: 50% to 170% IX: 60% to 136% X: 10% XI: 50% to 150% XII: 30% • Table 4-8 describes screening laboratory results in coagulation factor deficiencies.

COMPLEMENT Normal range: C3: 70-160 mg/dl C4: 20-40 mg/dl Abnormal values: DECREASED C3: Active SLE, immune complex disease, acute glomerulonephritis, inborn C3 deficiency, membranoproliferative glomerulonephritis, infective endocarditis, serum sickness, autoimmune/chronic active hepatitis

Characteristics of Coagulation Factors

Factor

Descriptive Name

Source

Approximate Half-Life (hr)

Function

I II V VII

Liver Liver (VKD) Liver Liver (VKD)

120 60 12-36 6

Substrate for fibrin clot (CP) Serine protease (CP) Cofactor (CP) (?) Serine protease (EP)

Endothelial cells and (?) elsewhere Liver (VKD)

12

Cofactor (IP)

24

Serine protease (IP)

Liver (VKD) (?) Liver

36 40-84

Serine protease (CP) Serine protease (IP)

XII

Fibrinogen Prothrombin Proaccelerin, labile factor Serum prothrombin conversion accelerator, proconvertin Antihemophilic factor or globulin Plasma thromboplastin component, Christmas factor Stuart-Prower factor Plasma thromboplastin antecedent Hageman factor

(?) Liver

50

XIII Prekallikrein

Fibrin-stabilizing factor Fletcher factor

(?) Liver (?) Liver

96-180 ?

High-molecularweight kininogen

Fitzgerald factor, Flaujeac or Williams factor

(?) Liver

?

Serine protease contact activation (IP) Transglutaminase (CP) Serine protease contact activation (IP) Cofactor, contact activation (IP)

VIII IX X XI

From Noble J (ed): Primary care medicine, ed 3, St Louis, 2001, Mosby. CP, Common pathway; EP, extrinsic pathway; IP, intrinsic pathway; VKD, vitamin K dependent.

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Complete Blood Count

TABLE 4-8

C-Reactive Protein, High Sensitivity

421

Screening Laboratory Results in Coagulation Factor Deficiencies

Deficient Factor

Frequency

PT

PTT

TT

I (fibrinogen) II (prothrombin) V 1:1,000,000 VII VIII IX X 1:500,000 XI XII† or HMWK† or PK† XIII

Rare Very rare ↑ 1:500,000 1:5000 (male) 1:30,000 (male) ↑ Rare* Rare Rare

↑ ↑ ↑ ↑ NL NL ↑ NL NL NL

↑ ↑ NL NL ↑ ↑ NL ↑ ↑ NL

↑ ↑ NL NL NL NL NL NL

From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders. ↑, Increased over normal range; HMWK, high-molecular-weight kininogen; NL, normal; PK, prekallikrein; PT, prothrombin time; PTT, partial thromboplastin time; TT, thrombin time. *Except in those of Ashkenazi Jewish descent (approximately 4% are heterozygous for factor XI deficiency). †Not associated with clinical bleeding.

DECREASED C4: Immune complex disease, active SLE, infective endocarditis, inborn C4 deficiency, hereditary angioedema, hypergammaglobulinemic states, cryobulinemic vasculitis Table 4-9 describes complement deficiency states.

COMPLETE BLOOD COUNT (CBC)

COOMBS, DIRECT Normal: Negative Positive: Autoimmune hemolytic anemia, transfusion reactions, drugs ( -methyldopa, penicillins, tetracycline, sulfonamides, levodopa, cephalosporins, quinidine, insulin) False positive: May be seen with cold agglutinins

COOMBS, INDIRECT Normal: Negative Positive: Acquired hemolytic anemia, incompatible crossmatched blood, anti-Rh antibodies, drugs (methyldopa, mefenamic acid, levodopa)

CORTISOL, PLASMA Normal range: Varies with time of collection (circadian variation): 8 AM: 4-19 g/dl (110-520 nmol/L) 4 PM: 2-15 g/dl (50-410 nmol/L) Elevated in: Ectopic adrenocorticotropic hormone production (i.e., oat cell carcinoma of lung), loss of normal diurnal variation, chronic renal failure, iatrogenic, stress, adrenal, or pituitary hyperplasia or adenomas Decreased in: Primary adrenocortical insufficiency, anterior pituitary hypofunction, secondary adrenocortical insufficiency, adrenogenital syndromes

C-PEPTIDE Elevated in: Insulinoma, sulfonylurea administration Decreased in: Insulin-dependent diabetes mellitus, factitious insulin administration

CPK; see CREATINE KINASE C-REACTIVE PROTEIN Normal range: 6.8-820 g/dl (68-8200 g/L) Elevated in: Rheumatoid arthritis, rheumatic fever, inflammatory bowel disease, bacterial infections, myocardial infarction, inflammatory and neoplastic diseases

C-REACTIVE PROTEIN, HIGH SENSITIVITY (hs-CRP, Cardio-CRP) is a new test used as a cardiac risk marker. It is increased in patients with silent atherosclerosis years before a cardiovascular event and is independent of cholesterol level and other lipoproteins. It can be used to help stratify cardiac risk.

LABORATORY TESTS AND INTERPRETATION

CONJUGATED BILIRUBIN; see BILIRUBIN, DIRECT

Normal range: 70-140 g/dl (11-22 mol/L) Decreased in: Wilson’s disease, malabsorption, malnutrition, nephrosis, total parenteral nutrition, acute leukemia in remission Elevated in: Aplastic anemia, biliary cirrhosis, systemic lupus erythematosus, hemochromatosis, hyperthyroidism, hypothyroidism, infection, iron deficiency anemia, leukemia, lymphoma, pernicious anemia, rheumatoid arthritis

Section IV

White blood cells 3200-9800 mm3 (3.2-9.8 109/L) Red blood cells Male: 4.3-5.9 106/mm3 (4.3-5.9 1012/L) Female: 3.5-5 106/mm3 (3.5-5 1012/L) Hemoglobin Male: 13.6-17.7 g/dl (136-172 g/L) Female: 12-15 g/dl (120-150 g/L) Hematocrit Male: 39% to 49% (0.39-0.49) Female: 33% to 43% (0.33-0.43) Mean corpuscular volume (MCV): 76-100 m3 (76-100 fL) Mean corpuscular hemoglobin (MCH): 27-33 pg (27-33 pg) Mean corpuscular hemoglobin concentration (MCHC): 33-37 g/dl (330-370 g/L) Red blood cell distribution width index (RDW): 11.5% to 14.5% Platelet count: 130-400 103/mm3 (130-400 109/L) Differential: 2-6 stabs (bands, early mature neutrophils) 60-70 segs (mature neutrophils) 1-4 eosinophils 0-1 basophils 2-8 monocytes 25-40 lymphocytes

COPPER (serum)

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Creatine Kinase

TABLE 4-9

Creatine Kinase

Complement Deficiency States Number of Reported Patients

Mode of Inheritance

Functional Defects

Disease Associations

31 21 109

ACD ACD ACD

Impaired IC handling, delayed C´ activation, impaired immune response

CVD, 48%; infection (encaps bact), 22%; both, 18%; healthy, 12%

3 70

ACD XL

Impaired C´ activation in absence of specific antibody

Infection (meningococcal), 74%; healthy, 26%

19

ACD

Impaired IC handling, opson/ phag; granulocytosis, CTX, immune response, and absent SBA

CVD, 79%; recurrent infection (encaps bact), 71%

27

ACD

Impaired CTX; absent SBA

77 73 73 165

ACD ACD ACD ACD

Absent SBA

Infection (Neisseria, primarily meningococcal), 58%; CVD, 4% Both, 1% Healthy, 25%

Impaired SBA

Healthy, 91%; infection, 9%

Many

AD Acq

Hereditary angioedema

H

13

ACD

Uncontrolled generation of an inflammatory mediator on C´ activation Uncontrolled AP activation → low C3

I

14

ACD

Uncontrolled AP activation → low C3

Many

Acq

Paroxysmal nocturnal hemoglobinuria

20

ACD

Impaired regulation of C3b and C8 deposited on host RBC; PMN, platelets → cell lysis Impaired PMN adhesive functions (i.e., margination), CTX, C3bi-mediated opson/phag

59

Acq

Stabilize AP, convertase → low C3

Acq

Stabilize CP, C3 convertase → low C3

MPGN, 41%; PLD, 25%; infection (encaps bact), 16%; MPGN plus PLD, 10%; PLD plus infection, 5%; MPGN plus PLD plus infection, 3%; MPGN plus infection, 2% Glomerulonephritis, 50%; CVD, 50%

Component Classic pathway C1qrs C4 C2 Alternative pathway D P Junction of classic and alternative pathways C3

Terminal components C5 C6 C7 C8 C9 Plasma proteins regulating C´ activation C1-INH

Membrane proteins regulating C´ activation Decay-accelerating factor Homologous restriction factor CD59 CR3

Autoantibodies C3 nephritic factors

C4 nephritic factor

CVD, 40%; CVD plus infection (encaps bact), 40%; healthy, 20% Infection (encaps bact), 100%

Infection (Staphylococcus aureus, Pseudomonas spp.), 100%

From Mandell GL: Mandell, Douglas, and Bennett’s principles and practice of infectious diseases, ed 5, New York, 2000, Churchill Livingstone. ACD, Autosomal codominant; Acq, acquired; AD, autosomal dominant; AP, alternative pathway; C´, complement; CP, classic pathway; CTX, chemotaxis; CVD, collagenvascular disease; encaps bact, encapsulated bacteria; IC, immune complex, MPGN, membranoproliferative glomerulonephritis; PLD, partial lipodystrophy; PMN, polymorphonuclear neutrophil; RBC, red blood cells; SBA, serum bactericidal activity; XL, X-linked.

Interpretation of results:

CREATINE KINASE (CK, CPK)

Cardio-CRP result (mg/L)

Normal range: 0-130 U/L Elevated in: Myocardial infarction, myocarditis, rhabdomyolysis, myositis, crush injury/trauma, polymyositis, dermatomyositis, vigorous exercise, muscular dystrophy, myxedema, seizures, malignant hyperthermia syndrome, IM injections, cerebrovascular accident, pulmonary embolism and infarction, acute dissection of aorta Decreased in: Steroids, decreased muscle mass, connective tissue disorders, alcoholic liver disease, metastatic neoplasms

0.6 0.7-1.1 1.2-1.9 2.0-3.8 3.9-4.9 5.0

RISK Lowest risk Low risk Moderate risk High risk Highest risk Results may be confounded by acute inflammatory disease. If clinically indicated, a repeat test should be performed in 2 or more weeks.

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Creatine Kinase Isoenzymes

Creatinine Clearance

423

Serum enzyme level

CPK

cTnI, cTnT

LDH AST Normal

5

10 Days after infarction

15

FIGURE 4-2 Evaluation of creatine kinase elevation. CPK, Creatine phosphokinase. (From Greene HL, Johnson WP, Lemcke D [eds]: Decision making in medicine, ed 2, St Louis, 1998, Mosby.)

Normal range: 0.6-1.2 mg/dl Elevated in: Renal insufficiency (acute and chronic), decreased renal perfusion (hypotension, dehydration, congestive heart failure), urinary tract infection, rhabdomyolysis, ketonemia Drugs (antibiotics [aminoglycosides, cephalosporins], hydantoin, diuretics, methyldopa) Falsely elevated in: Diabetic ketoacidosis, administration of some cephalosporins (e.g., cefoxitin, cephalothin) Decreased in: Decreased muscle mass (including amputees and older persons), prolonged debilitation

CREATININE CLEARANCE Normal range: 75-124 ml/min. Box 4-1 describes a formula for calculation of creatinine clearance. The Cockcroft-Gault formula to calculate creatinine clearance is described in Box 4-2. Elevated in: Exercise Decreased in: Renal insufficiency, drugs (cimetidine, procainamide, antibiotics, quinidine)

BOX 4-1

Calculation of the Creatinine Clearance

BOX 4-2

Ccr Ucr V/Pcr where Ccr clearance of creatinine (ml/min) Ucr urine creatinine (mg/dl) V volume of urine (ml/min) (for 24-hr volume: divide by 1440) Pcr plasma creatinine (mg/dl)

(140  age in year) (lean body weight in kg) Ccr



Scr in mg/dl  72

Normal range: 95 to 105 ml/min/1.75 m2

Cockroft-Gault Formula to Calculate Creatinine Clearance (Ccr)

For women multiply final value by 0.85

LABORATORY TESTS AND INTERPRETATION

CREATININE (serum)

CK-BB: Elevated in: cerebrovascular accident, subarachnoid hemorrhage, neoplasms (prostate, gastrointestinal tract, brain, ovary, breast, lung), severe shock, bowel infarction, hypothermia, meningitis CK-MB: Elevated in: myocardial infarction (MI), myocarditis, pericarditis, muscular dystrophy, cardiac defibrillation, cardiac surgery, extensive rhabdomyolysis, strenuous exercise (marathon runners), mixed connective tissue disease, cardiomyopathy, hypothermia NOTE: CK-MB exists in the blood in two subforms. MB2 is released from cardiac cells and converted in the blood to MB1. Rapid assay of CK-MB subforms can detect MI (CK-MB2 1.0 U/L, with a ratio of CK-MB2/CK-MB1 1.5) within 6 hours of onset of symptoms. Fig. 4-2 illustrates the time course of CK, AST, troponins, and LDH activity after acute MI. CK-MM: Elevated in: crush injury, seizures, malignant hyperthermia syndrome, rhabdomyolysis, myositis, polymyositis, dermatomyositis, vigorous exercise, muscular dystrophy, IM injections, acute dissection of aorta

Section IV

CREATINE KINASE ISOENZYMES

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Cryoglobulins CRYOGLOBULINS (serum)

ENA-COMPLEX; see EXTRACTABLE NUCLEAR ANTIGEN

Normal range: Not detectable Present in: Collagen-vascular diseases, chronic lymphocytic leukemia, hemolytic anemias, multiple myeloma, Waldenström’s macroglobulinemia, chronic active hepatitis, Hodgkin’s disease

D-DIMER

Normal range: 0.3 mcg/mL Elevated in: DVT, pulmonary embolism, high levels of rheumatoid factor, activation of coagulation and fibrolytic system from any cause D-dimer assay by ELISA assists in the diagnosis of DVT and pulmonary embolism. This test has significant limitations because it can be elevated whenever the coagulation and fibrinolytic systems are activated and can also be falsely elevated with high rheumatoid factor levels.

ABSORPTION

Normal range: 21% to 31% excreted in 5 hr Decreased in: Malabsorption syndrome D-XYLOSE

ENDOMYSIAL ANTIBODIES Normal: Not detected Present in: Celiac disease, dermatitis herpetiformis

EPSTEIN-BARR VIRUS SEROLOGY

CSF; see CEREBROSPINAL FLUID

D-XYLOSE

Erythropoietin

ABSORPTION TEST

Normal range: URINE:  4 g/5 hours (5-hour urine collection in adults  12 years (25 g dose) SERUM:  25 mg/dl (adult, 1 h, 25 g dose, normal renal function) Normal results: In patients with malabsorption, normal results suggest pancreatic disease as an etiology of the malabsorption. Abnormal results: Celiac disease, Crohn’s disease, tropical sprue, surgical bowel resection, AIDS. False-positives can occur with decreased renal function, dehydration/hypovolemia, surgical blind loops, decreased gastric emptying, vomiting.

ELECTROPHORESIS, HEMOGLOBIN; see HEMOGLOBIN ELECTROPHORESIS ELECTROPHORESIS, PROTEIN; see PROTEIN ELECTROPHORESIS

Normal range: IgG anti VCA 1:10 or negative Abnormal: IgG anti VCA 1:10 or positive indicates either current or previous infection IgM anti VCA 1:10 or positive indicates current or recent infection Anti-EBNA 1.5 or positive indicates previous infection Table 4-10 and Fig. 4-3 describe test interpretation.

ERYTHROCYTE SEDIMENTATION RATE (ESR; Westergren) Normal range: Male: 0-15 mm/hr Female: 0-20 mm/hr Elevated in: Collagen-vascular diseases, infections, myocardial infarction, neoplasms, inflammatory states (acute phase reactant), hyperthyroidism, hypothyroidism, rouleaux formation Decreased in: Sickle cell disease, polycythemia, corticosteroids, spherocytosis, anisocytosis, hypofibrinogenemia, increased serum viscosity

ERYTHROPOIETIN (EP) Normal: 3.7-16.0 IU/L by radioimmunoassay Erythropoietin is a glycoprotein secreted by the kidneys that stimulates RBC production by acting on erythroid-committed stem cells. Increased in: Extremely high: generally seen in patients with severe anemia (Hct 25, 7) such as in cases of aplastic anemia, severe hemolytic anemia, hematologic cancers. Very high: patients with mild to moderate anemia (Hct 25-35, Hb 7-10); high: patients with mild anemia (e.g., AIDS, myelodysplasia). Erythropoietin can be inappropriately elevated in patients with malignant neoplasms, renal cysts, postrenal transplant, meningioma, hemangioblastoma, and leiomyoma. Decreased in: Renal failure, polycythemia vera, autonomic neuropathy

Clinical illness

Incubation

Heterophil EBNA-IgG

VCA-IgM VCA-IgG EA-D

0

1

2

3

4

5

6

7

8

9

10

11

12

13

14

15

16

17

Wk after infection FIGURE 4-3 Tests in Epstein-Barr viral infection. See Table 4-10 for abbreviations. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

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Estradiol

TABLE 4-10

Free Thyroxine Index

425

Antibody Tests in Epstein-Barr Viral Infection

Heterophil Ab VCA-IgM VCA-IgG EBNA-IgG EA-D (EBNA-IgM)

Appearance

Peak

Disappears

3-5 days after onset of Sx (range, 0-21 days) Beginning of Sx (1 wk before to 1 wk after Sx begins) 3 days after onset of Sx (0-2 wk) 3 wk after onset of Sx (1-4 wk) 5 days after onset of Sx (during first 1-2 wk after onset of Sx) (Same as VCA-IgM)

During second wk after onset of Sx (1-4 wk) During first wk after onset of Sx (0-21 days) During second wk after onset of Sx (1-3 wk) 8 mo after appearance (3-12 mo) 14-21 days after onset of Sx (1-4 wk)

2-3 mo after onset of Sx (still found at 1 yr in 20% of cases) 2-3 mo after onset of Sx (1-6 mo) Decline to lower level, then persists for life Lifelong 9 wk after appearance (2-6 mo)

(Same as VCA-IgM)

(Same as VCA-IgM)

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. Ab, Antibody; EA, early antigen; EBNA, Epstein-Barr virus nuclear antigen; Sx, symptoms; VCA, viral capsid antigen.

ESTRADIOL (serum)

-1 FETOPROTEIN

Normal range: FEMALE, POSTMENOPAUSAL: 0-30 pg/ml MALE, ADULT: 10-50 pg/ml Decreased in: Ovarian failure Elevated in: Tumors of ovary, testis, adrenal, or nonendocrine sites (rare)

Normal range: 0-20 ng/ml Elevated in: Hepatocellular carcinoma (usually values 1000 ng/ml), germinal neoplasms (testis, ovary, mediastinum, retroperitoneum), liver disease (alcoholic cirrhosis, acute hepatitis, chronic active hepatitis), basal cell carcinoma, breast carcinoma, pancreatic carcinoma, gastric carcinoma, esophageal atresia

ESTROGEN Normal range: Serum: Males: Females:

20-80 pg/ml Follicular: 60-200 pg/ml Luteal: 160-400 pg/ml Postmenopausal: 130 pg/ml Urine: Males: 4-23 g/g creatinine Females: Follicular: 7-65 g/g creatinine Midcycle: 32-104 g/g creatinine Luteal: 8-135 g/g creatinine Elevated in: Hyperplasia of adrenal cortex, ovarian tumors producing estrogen, granulosa and thecal cell tumors, testicular tumors Decreased in: Menopause

ETHANOL (blood)

EXTRACTABLE NUCLEAR ANTIGEN (ENA complex, anti-RNP antibody, anti-Sm, anti-Smith)

FECAL FAT, QUANTITATIVE (72-hr collection) Normal range: 2-6 g/24 hr Elevated in: Malabsorption syndrome

FERRITIN (serum) Normal range: 18-300 ng/ml Elevated in: Hyperthyroidism, inflammatory states, liver disease (ferritin elevated from necrotic hepatocytes), neoplasms (neuroblastomas, lymphomas, leukemia, breast carcinoma), iron replacement therapy, hemochromatosis, hemosiderosis Decreased in: Iron deficiency anemia

FREE T4 ; see T4, FREE FREE THYROXINE INDEX Normal range: 1.1-4.3 INCREASED THYROXINE OR FREE THYROXINE VALUES Laboratory error Primary hyperthyroidism (T4/T3 type) Severe thyroxine-binding globulin elevation Excess therapy of hypothyroidism Excessive dose of levothyroxine Active thyroiditis (subacute, painless, early active Hashimoto’s disease) Familial dysalbuminemic hyperthyroxinemia (some FT4 kits, especially analog types) Peripheral resistance to T4 syndrome Amiodarone or propranolol Factitious hyperthyroidism Jod-Basedow (iodine-induced) hyperthyroidism Severe nonthyroid illness T4 sample drawn 2-4 hr after levothyroxine dose Struma ovarii Pituitary thyroid-stimulating hormone–secreting tumor Certain x-ray contrast media (Telepaque and Oragrafin) Acute porphyria Heparin effect (some T4 and FT4 kits) Amphetamine, heroin, methadone, and phencyclidine abuse Perphenazine or 5-fluorouracil Antithyroid or anti-IgG heterophil (HAMA) autoantibodies “T4” hyperthyroidism

LABORATORY TESTS AND INTERPRETATION

Normal: Negative Present in: Systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome, mixed connective tissue disease

Normal range: Plasma: 2-10 ng/ml Red blood cells: 140-960 ng/ml Decreased in: Folic acid deficiency (inadequate intake, malabsorption), alcoholism, drugs (methotrexate, trimethoprim, phenytoin, oral contraceptives, Azulfidine), vitamin B12 deficiency (defective red cell folate absorption), hemolytic anemia Elevated in: Folic acid therapy

Section IV

Normal range: Negative (values 10 mg/dl are considered negative) Ethanol is metabolized at 10-25 mg/dl/hour. Levels 80 mg/dl are considered evidence of impairment for driving. Fatal blood concentration is considered to be 400 mg/dl.

FOLATE (folic acid)

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Glucose, Fasting Hyperemesis gravidarum; about 50% of patients High altitudes DECREASED THYROXINE OR FREE THYROXINE VALUES Laboratory error Primary hypothyroidism Severe nonthyroid illness* Lithium therapy Severe thyroxine-binding globulin decrease (congenital, disease, or drug-induced) or severe albumin decrease* Dilantin, Depakene, or high-dose salicylate drugs* Pituitary insufficiency Large doses of inorganic iodide (e.g., saturated solution of potassium iodide) Moderate or severe iodine deficiency Cushing’s syndrome High-dose glucocorticoid drugs Addison’s disease; some patients (30%) Heparin effect (a few FT4 kits) Desipramine or amiodarone drugs Acute psychiatric illness

GLUCOSE, FASTING Normal range: 70-110 mg/dl Elevated in: Diabetes mellitus, stress, infections, myocardial infarction, cerebrovascular accident, Cushing’s syndrome, acromegaly, acute pancreatitis, glucagonoma, hemochromatosis, drugs (glucocorticoids, diuretics [thiazides, loop diuretics]), glucose intolerance Decreased in: Sulfonylurea therapy, insulin therapy, reactive hypoglycemia (e.g., s/b subtotal gastrectomy), starvation, insulinoma, glycogen storage disorders, severe liver disease or renal disease, ethanol-induced hypoglycemia, mesenchymal tumors that secrete insulin-like hormones

GLUCOSE, POSTPRANDIAL Normal range: 140 mg/dl Elevated in: Diabetes mellitus, glucose intolerance Decreased in: Postgastrointestinal resection, reactive hypoglycemia

GLUCOSE TOLERANCE TEST Normal values above fasting: 30 min: 30-60 mg/dl 60 min: 20-50 mg/dl 120 min: 5-15 mg/dl 180 min: fasting level or below Abnormal in: Glucose intolerance, diabetes mellitus, Cushing’s syndrome, acromegaly, pheochromocytoma

-GLUTAMYL TRANSFERASE (GGT) Normal range: 0-30 U/L Elevated in: Chronic alcoholic liver disease, neoplasms (hepatoma, metastatic disease to the liver, carcinoma of the pancreas), systemic lupus erythematosus, congestive heart failure, trauma, nephrotic syndrome, sepsis, cholestasis, drugs (phenytoin, barbiturates)

GLYCATED (GLYCOSYLATED) HEMOGLOBIN (HbA1C) Normal range: 4.0% to 6.7% Elevated in: Uncontrolled diabetes mellitus (glycated hemoglobin levels reflect the level of glucose control over the preceding 120 days), lead toxicity, alcoholism, iron deficiency anemia, hypertriglyceridemia Decreased in: Hemolytic anemias, decreased red blood cell survival, acute or chronic blood loss, chronic renal failure, insulinoma, congenital spherocytosis, hemoglobin S, C, and D diseases

●

Hepatitis A Antibody HAM TEST (acid serum test) Normal: Negative Positive in: Paroxysmal nocturnal hemoglobinuria False positive in: Hereditary or acquired spherocytosis, recent transfusion with aged red blood cells, aplastic anemia, myeloproliferative syndromes, leukemia, hereditary dyserythropoietic anemia type II

HAPTOGLOBIN (serum) Normal range: 50-220 mg/dl Elevated in: Inflammation (acute phase reactant), collagen-vascular diseases, infections (acute phase reactant), drugs (androgens), obstructive liver disease Decreased in: Hemolysis (intravascular more than extravascular), megaloblastic anemia, severe liver disease, large tissue hematomas, infectious mononucleosis

HDL; see HIGH-DENSITY LIPOPROTEIN CHOLESTEROL HELICOBACTER PYLORI (serology, stool antigen) Normal range: Not detected Detected in: H. pylori infection. Positive serology can indicate current or past infection. Positive stool antigen test indicates acute infection (sensitivity and specificity >90%). Stool testing should be delayed at least 4 weeks after eradication therapy.

HEMATOCRIT Normal range: Male: 39% to 49% Female: 33% to 43% Elevated in: Polycythemia vera, smoking, chronic obstructive pulmonary disease, high altitudes, dehydration, hypovolemia Decreased in: Blood loss (gastrointestinal, genitourinary) anemia

HEMOGLOBIN Normal range: Male: 13.6-17.7 g/dl Female: 12.0-15.0 g/dl Elevated in: Hemoconcentration, dehydration, polycythemia vera, chronic obstructive pulmonary disease, high altitudes, false elevations (hyperlipemic plasma, white blood cells 50,000/mm3), stress Decreased in: Hemorrhage (gastrointestinal, genitourinary) anemia

HEMOGLOBIN A1C, see GLYCATED HEMOGLOBIN HEMOGLOBIN ELECTROPHORESIS Normal range: HbA1: 95%-98% HbA2: 1.5%-3.5% HbF: 2% HbC: absent HbS: absent

HEMOGLOBIN, GLYCATED; see GLYCATED HEMOGLOBIN HEMOGLOBIN, GLYCOSYLATED; see GLYCATED HEMOGLOBIN HEPATITIS A ANTIBODY Normal: Negative Present in: Viral hepatitis A; can be IgM or IgG (if IgM, acute hepatitis A; if IgG, previous infection with hepatitis A) See Fig. 4-4 for serologic tests in HAV infection. HAV-IGM ANTIBODY Appearance About the same time as clinical symptoms (3-4 wk after exposure, range 14-60 days), or just before beginning of AST/ALT elevation (range 10 days before–7 days after)

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Hepatitis A Viral Infection

Hepatitis B Viral Infection

427

Transaminases Clinical symptoms

HAV-Ab (total)

HAV-Ag in stool

0

5

10

HAV-Ab (IgM)

15

20

25

30

35

40

45

50

55

60

Days after exposure

FIGURE 4-4 Serologic tests in HAV infection. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

Peak About 3-4 wk after onset of symptoms (1-6 wk) Becomes Nondetectable 3-4 mo after onset of symptoms (1-6 mo). In a few cases HAVIgM antibody can persist as long as 12-14 mo. HAV-TOTAL ANTIBODY Appearance About 3 wk after IgM becomes detectable (therefore about the middle of clinical symptom period to early convalescence) Peak About 1-2 mo after onset Becomes Nondetectable Remains elevated for life, but can slowly fall somewhat

HEPATITIS A VIRAL INFECTION Best all-purpose test(s) to diagnose acute HAV infection HAVAb (IgM) Best all-purpose test(s) to demonstrate past HAV infection/immunity HAV-Ab (total) Normal: Not detected Detected in: Acute viral hepatitis type B, chronic hepatitis B Appearance

Peak

1-2 wk before to 1-2 wk after onset of symptoms Becomes Nondetectable

1-3 mo after peak (range 1 wk-5 mo)

HEPATITIS B VIRAL INFECTION Figs. 4-5, 4-6, and 4-7 illustrate antigens and antibodies in hepatitis B infection. HBS -Ag HBSAg: shows current active HBV infection. Persistence over 6 mo indicates carrier/chronic HBV infection.

LABORATORY TESTS AND INTERPRETATION

2-6 wk after exposure (range 6 days–6 mo); 5%-15% of patients are negative at onset of jaundice

Section IV

HEPATITIS B SURFACE ANTIGEN (HBSAG)

HBV nucleic acid probe: present before and longer than HBSAg. More reliable marker for increased infectivity than HBSAg and/or HBeAg. -Ab HBSAb-total: shows previous healed HBV infection and evidence of immunity. HBC -Ab HBCAb-IgM: shows either acute or very recent infection by HBV. In convalescent phase of acute HBV, may be elevated when HBSAg has disappeared (core window). Negative HBCAb-IgM with positive HBSAg suggests either very early acute HBV or carrier/chronic HBV. HBCAb-total: only useful to show past HBV infection if HBSAg and HBcAb-IgM are both negative. HBE -Ag HBe-AbAg: when present, especially without HBeAb, suggests increased patient infectivity. HBeAb-total: when present, suggests less patient infectivity. I. HBSAg positive, HBCAb negative* About 5% (range 0%-17%) of patients with early-stage HBV acute infection (HBCAb rises later) II. HBSAg positive, HBCAb positive, HBSAb negative a. Most of the clinical symptom stage b. Chronic HBV carriers without evidence of liver disease (“asymptomatic carriers”) c. Chronic HBV hepatitis (chronic persistent type or chronic active type) III. HBSAg negative, HBCAb positive,* HBSAb negative a. Late clinical symptom stage or early convalescence stage (core window) b. Chronic HBV infection with HBSAg below detection levels with current tests c. Old previous HBV infection IV. HBSAg negative, HBCAb positive, HBSAb positive a. Late convalescence to complete recovery b. Old infection

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Hepatitis B Viral Infection

Hepatitis B Viral Infection

AST (SGOT) elevation Clinical symptoms Jaundice HBcAb*

HBcAbIgM

HBsAb

HBsAg

0

1

2

3

4

5

6

12

24

Mo after exposure

FIGURE 4-5 HBV surface antigen-antibody and core antibodies (note “core window”). *HBCAb HBCAb-IgM  HBCAb-IgG (combined). (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

AST (SGOT) elevation Clinical symptoms Jaundice

HBsAb

HBsAg

0

1

2

3

4

5

6

12

24

Mo after exposure

FIGURE 4-6 HBV surface antigen and antibody (HBSAg and HBSAb-total). (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

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Hepatitis C Viral Infection

Hepatitis C Viral Infection

429

AST (SGOT) elevation Clinical symptoms Jaundice

HBeAb (total)

HBeAg

0

1

2

3

4

5

6

12

24

Mo after exposure

FIGURE 4-7 HBV e antigen and antibody. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

HEPATITIS C VIRAL INFECTION Fig. 4-8 illustrates antigens and antibodies in hepatitis C infection. HCV -Ag HCV nucleic acid probe: shows current infection by HCV (especially using PCR amplification). -Ab HCV-Ab (IgG): current, convalescent, or old HCV infection.

HAV -Ag HAV-Ag by EM: shows presence of virus in stool early in infection. -Ab HAV-Ab (IgM): current or recent HAV infection. HAV-Ab (total): convalescent or old HAV infection.

AST (SGOT) elevation

Section IV

Clinical symptoms HCV-Ab (IgG)

Jaundice

0

1

2

3

4

5

6

12

24

Mo after exposure

FIGURE 4-8 HCV antigen and antibody. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

LABORATORY TESTS AND INTERPRETATION

HCV-Ag (RNA probe)

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High-Density Lipoprotein (HDL) Cholesterol

●

Lactate Dehydrogenase Isoenzymes

HIGH-DENSITY LIPOPROTEIN (HDL) CHOLESTEROL

INTERNATIONAL NORMALIZED RATIO (INR)

Normal range: Male: 45-70 mg/dl Female: 45-90 mg/dl Increased in: Use of gemfibrozil, statins, fenofibrate, nicotinic acid, estrogens, regular aerobic exercise, small (1 oz) daily alcohol intake Decreased in: Deficiency of apoproteins, liver disease, probucol ingestion, Tangier disease NOTE: A cholesterol/HDL ratio 4.0 is associated with increased risk of coronary artery disease.

The INR is a comparative rating of prothrombin time (PT) ratios. The INR represents the observed PT ratio adjusted by the International Reference Thromboplastin. It provides a universal result indicative of what the patient’s PT result would have been if measured using the primary World Health Organization International Reference reagent. For proper interpretation of INR values, the patient should be on stable anticoagulant therapy. Recommended INR ranges: Proximal deep vein thrombosis: 2-3 Pulmonary embolism: 2-3 Transient ischemic attacks: 2-3 Atrial fibrillation: 2-3 Mechanical prosthetic valves: 3-4.5 Recurrent venous thromboembolic disease: 3-4.5

5-HYDROXYINDOLE-ACETIC ACID, URINE; see URINE 5-HYDROXYINDOLE-ACETIC ACID IMMUNOGLOBULINS Normal range: IgA: 50-350 mg/dl IgD: 6 mg/dl IgE: 25 g/dl IgG: 800-1500 mg/dl IgM: 45-150 mg/dl Elevated in: IgA: lymphoproliferative disorders, Berger’s nephropathy, chronic infections, autoimmune disorders, liver disease IgE: allergic disorders, parasitic infections, immunologic disorders, IgE myeloma IgG: chronic granulomatous infections, infectious diseases, inflammation, myeloma, liver disease IgM: primary biliary cirrhosis, infectious diseases (brucellosis, malaria), Waldenström’s macroglobulinemia, liver disease Decreased in: IgA: nephrotic syndrome, protein-losing enteropathy, congenital deficiency, lymphocytic leukemia, ataxia-telangiectasia, chronic sinopulmonary disease IgE: hypogammaglobulinemia, neoplasm (breast, bronchial, cervical), ataxia-telangiectasia IgG: congenital or acquired deficiency, lymphocytic leukemia, phenytoin, methylprednisolone, nephrotic syndrome, protein-losing enteropathy IgM: congenital deficiency, lymphocytic leukemia, nephrotic syndrome

TABLE 4-11

TIBC↓

SI↓ SI↑

TIBC↑ TIBC↓

SI↑

TIBC↑

SI↑

TIBC NL TIBC NL TIBC↑

SI NL

Normal range: 250-460 g/dl Elevated in: Iron deficiency anemia, polycythemia, hepatitis, weight loss Decreased in: Anemia of chronic disease, hemochromatosis, chronic liver disease, hemolytic anemias, malnutrition (protein depletion) Table 4-11 describes TIBC and serum iron abnormalities.

LACTATE DEHYDROGENASE (LDH) Normal range: 50-150 U/L Elevated in: Infarction of myocardium, lung, kidney Diseases of cardiopulmonary system, liver, collagen, central nervous system Hemolytic anemias, megaloblastic anemias, transfusions, seizures, muscle trauma, muscular dystrophy, acute pancreatitis, hypotension, shock, infectious mononucleosis, inflammation, neoplasia, intestinal obstruction, hypothyroidism

LACTATE DEHYDROGENASE ISOENZYMES Normal range: LDH1: 22% to 36% (cardiac, red blood cell) LDH2: 35% to 46% (cardiac, red blood cell) LDH3: 13% to 26% (pulmonary) LDH4: 3% to 10% (striated muscle, liver) LDH5: 2% to 9% (striated muscle, liver)

Serum Iron and Total Iron-Binding Capacity Patterns

SI↓

SI↓

IRON-BINDING CAPACITY, TOTAL (TIBC)

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. NL, Normal; SI, serum iron; TIBC, total iron-binding capacity.

Chronic diseases Uremia Chronic iron deficiency anemia Hemachromatosis Iron therapy overload (TIBC may be normal) Hemolytic anemia; thalassemia; lead poisoning; megaloblastic anemia; aplastic, pyridoxine deficiency, or other sideroblastic anemias Oral contraceptives Acute hepatitis (some report TIBC is low normal) Chronic hepatitis (some patients) B12 or folate deficiency Chronic iron deficiency (some patients) Acute infection, surgery, tissue damage B12/folate deficiency plus iron deficiency

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LAP Score Normal ratios: LDH1 LDH2 LDH5 LDH4 Abnormal values: LDH1 LDH2: myocardial infarction (can also be seen with hemolytic anemias, pernicious anemia, folate deficiency, renal infarct) LDH5 LDH4: liver disease (cirrhosis, hepatitis, hepatic congestion)

LAP SCORE; see LEUKOCYTE ALKALINE PHOSPHATASE LDH; see LACTATE DEHYDROGENASE LDL; see LOW-DENSITY LIPOPROTEIN CHOLESTEROL LEGIONELLA TITER Normal: Negative Positive in: Legionnaire’s disease (presumptive: 1:256 titer; definitive: fourfold titer increase to 1:128)

LEUKOCYTE ALKALINE PHOSPHATASE Normal range: 13-100 Elevated in: Leukemoid reactions, neutrophilia secondary to infections (except in sickle cell crisis—no significant increase in LAP score), Hodgkin’s disease, polycythemia vera, hairy cell leukemia, aplastic anemia, Down’s syndrome, myelofibrosis Decreased in: Acute and chronic granulocytic leukemia, thrombocytopenic purpura, paroxysmal nocturnal hemoglobinuria, hypophosphatemia, collagen disorders

LEUKOCYTE COUNT; see COMPLETE BLOOD COUNT LIPASE Normal range: 0-160 U/L Elevated in: Acute pancreatitis, perforated peptic ulcer, carcinoma of pancreas (early stage), pancreatic duct obstruction, bowel infarction, intestinal obstruction

LIPOPROTEIN CHOLESTEROL, HIGH-DENSITY; see HIGH-DENSITY LIPOPROTEIN CHOLESTEROL

Lymphocytes

431

LOW-DENSITY LIPOPROTEIN (LDL) CHOLESTEROL Normal range: 50-130 mg/dl LDL cholesterol 100 Optimal 100-129 Near or above optimal 130-159 Borderline high 160-189 High 190 Very high

LUPUS ANTICOAGULANT; see CIRCULATING ANTICOAGULANT LYME DISEASE ANTIBODY TITER Normal range: Negative Positive result: Fig. 4-9 illustrates the usual serologic response in Lyme disease. A serologic test is not necessary or helpful for several days after a tick bite, because it is only 40%-50% sensitive in this stage and a negative test does not rule out the diagnosis.

LYMPHOCYTES Normal range: 15% to 40%:

Total lymphocyte count 800-2600/mm3 Total T lymphocyte 800-2200/mm3 CD4 lymphocytes 400/mm3 CD8 lymphocytes 200-800/mm3 Normal CD4/CD8 ratio is 2.0 Elevated in: Chronic infections, infectious mononucleosis and other viral infections, chronic lymphocytic leukemia, Hodgkin’s disease, ulcerative colitis, hypoadrenalism, idiopathic thrombocytopenia Decreased in: AIDS, bone marrow suppression from chemotherapeutic agents or chemotherapy, aplastic anemia, neoplasms, steroids, adrenocortical hyperfunction, neurologic disorders (multiple sclerosis, myasthenia gravis, Guillain-Barré syndrome) CD4 lymphocytes are calculated as total white blood cells % lymphocytes % lymphocytes stained with CD4. They are decreased in AIDS and other immune dysfunction. Table 4-12 describes various lymphocyte abnormalities in peripheral blood.

LIPOPROTEIN CHOLESTEROL, LOW-DENSITY; see LOW-DENSITY LIPOPROTEIN CHOLESTEROL Section IV LABORATORY TESTS AND INTERPRETATION

FIGURE 4-9 IgM and IgG response in Lyme disease. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

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Magnesium

TABLE 4-12

Magnesium

Differential Diagnosis of Abnormal Lymphocytes in Peripheral Blood

Lymphocyte Type

Usual Disease Association

Cytologic Features

Laboratory Features

Clinical Features

Small lymphocyte

Chronic lymphocytic leukemia

B-cell surface markers with low concentration of surface immunoglobulin, CD5 antigen

Hypogammaglobulinemia in 50%; positive direct Coombs’ test in 15%; on node biopsy, diffuse, well-differentiated lymphocytic infiltrate

Atypical lymphocyte

Infectious mononucleosis, other viral illnesses

Suppressor T-cell markers

Plasmacytoid lymphocyte

Waldenström’s macroglobulinemia

Cytoplasmic IgM, periodic acid–Schiff (PAS) positivity

Heterophil agglutinin; positive serology for Epstein-Barr virus, cytomegalovirus, toxoplasma, HBsAg IgM paraprotein, rouleaux, cryoglobulins

Elderly adults; presentation runs gamut from asymptomatic with lymphocytosis only to bulky disease with adenopathy, splenomegaly, and “packed” bone marrow Pharyngitis, fever, adenopathy, rash, splenomegaly, palatal petechiae, jaundice

Lymphoblast

Acute lymphoblastic leukemia (ALL)

Lymphosarcoma cell

Lymphocytic lymphoma

Terminal transferase positivity, common ALL antigen, B- or T-precursor markers B-cell surface markers with high concentration of monoclonal surface immunoglobulin

Sézary cell

Cutaneous lymphomas

T-lymphocyte surface markers

Hairy cell

Hairy cell leukemia

Prolymphocyte

Prolymphocytic leukemia

B-lymphocyte markers, cytoplasmic projections, tartrate-resistant acid phosphatase, interleukin-2 receptors, CD11 antigen B-cell surface markers with high concentration of surface immunoglobulin, CD5 negative

Anemia, granulocytopenia, thrombocytopenia, hyperuricemia, diffuse bone marrow infiltration Nodular or diffuse, poorly differentiated lymphocytic lymphoma on node biopsy, patchy, peritrabecular bone marrow involvement Skin biopsy is diagnostic

Pancytopenia

Marked lymphocytosis (frequently 100 109/L)

From Stein JH (ed): Internal medicine, ed 5, St Louis, 1998, Mosby.

MAGNESIUM (serum) Normal range: 1.8-3.0 mg/dl CAUSES OF HYPERMAGNESEMIA I. Decreased renal excretion A. Renal failure—glomerular filtration rate less than 30 ml/min B. Hyperparathyroidism C. Hypothyroidism D. Addison’s disease E. Lithium intoxication F. Familial hypocalciuric hypercalcemia II. Other causes: usually in association with decrease in glomerular filtration rate A. Endogenous loads 1. Diabetic ketoacidosis 2. Severe tissue injury—burns B. Exogenous loads 1. Gastrointestinal a. Magnesium-containing laxatives and antacids b. High-dose vitamin D analogs

CAUSES OF HYPOMAGNESEMIA Alcohol abuse Diuretic use Renal losses Acute and chronic renal failure Postobstructive diuresis Acute tubular necrosis Chronic glomerulonephritis Chronic pyelonephritis Interstitial nephropathy Renal transplantation Gastrointestinal losses Chronic diarrhea Nasogastric suctioning Short bowel syndrome Protein calorie malnutrition Bowel fistula Total parenteral nutrition Acute pancreatitis Endocrine

Adenopathy, splenomegaly, absence of bone lesions, hyperviscosity syndrome, cryopathic phenomena Peak incidence in childhood, acute onset, bone pain frequent Middle-aged to older adults, generalized adenopathy, constitutional symptoms

Exfoliative erythroderma, cutaneous plaques or tumors Middle-aged males, moderate to marked splenomegaly without adenopathy

Elderly adults, massive splenomegaly, minimum adenopathy, poor response to therapy

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Mean Corpuscular Volume Diabetes mellitus Hyperaldosteronism Hyperthyroidism Hyperparathyroidism Acute intermittent porphyria Drugs Aminoglycosides Amphotericin -Agonists Cisplatin Cyclosporine Diuretics Foscarnet Pentamidine Theophylline Familial hypomagnesemia Maternal hypothyroidism Maternal hyperparathyroidism

TABLE 4-13

Neutrophil Count MEAN CORPUSCULAR VOLUME (MCV) Normal range: 76-100 m3 (76-100 fL) See Tables 4-13 and 4-14 for descriptions of MCV abnormalities.

NEUTROPHIL COUNT Normal range: 50% to 70% Subsets Stabs (bands, early mature neutrophils): 2% to 6% Segs (mature neutrophils): 60% to 70% Elevated in: Acute bacterial infections, acute myocardial infarction, stress, neoplasms, myelocytic leukemia Decreased in: Viral infections, aplastic anemias, immunosuppressive drugs, radiation therapy to bone marrow, agranulocytosis, drugs (antibiotics, antithyroidals, clopidogrel), lymphocytic and monocytic leukemias • Table 4-15 describes various drugs that can cause neutropenia.

Some Causes of Increased Mean Corpuscular Volume (Macrocytosis)

Causes

% of all Macrocytosis Patients*

% of Macrocytosis in Each Disease†

20-30 (5-50)‡ 15-20 (6-28) 10-12 (3-15) 10-15 (2-20) 8 (7-9.5) 6-7 (0-15) Frequent over age 40 yr 25 (22.5-27) 4%

80-90 (4-100) 25-30 (8-65) 60 (26-90) 30-40 (13-82) ? Depends on severity 60 in RAEB and RARS —

30 (14-50) 20-30 (8-55)

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby. RAEB, Refractory anemia with excessive blasts; RARS, refractory anemia with ring sideroblasts (formerly called IASA, or idiopathic acquired sideroblastic anemia). *Percentage of all patients with macrocytosis. †Percentage of patients with each condition listed who have macrocytosis. ‡Numbers in parentheses are literature range.

Common

Less Common

Chronic iron deficiency

- or -thalassemia (minor) Anemia of chronic disease

Some cases of polycythemia Some cases of lead poisoning Some cases of congenital spherocytosis Some cases of sideroblastic anemia Certain abnormal Hbs (Hb E, Hb Lepore)

From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.

LABORATORY TESTS AND INTERPRETATION

Some Causes of Decreased Mean Corpuscular Volume (Microcytosis)

Section IV

Common Folate or B12 deficiency Chronic liver disease Chronic alcoholism Cytotoxic chemotherapy Cardiorespiratory abnormality Reticulocytosis Myelodysplastic syndromes Unexplained Less Common Noncytotoxic drugs Zidovudine Phenytoin Azathioprine Hypothyroidism Chronic leukemia/myelofibrosis Radiotherapy for malignancy Chronic renal disease (occasional patients) Distance-runner macrocytosis (some persons) Down syndrome Artifactual (e.g., cold agglutinins)

TABLE 4-14

433

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434

Norepinephrine

TABLE 4-15

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Partial Thromboplastin Time

Drugs That Cause Neutropenia

Antiarrhythmics Tocainide, procainamide, propranolol, quinidine Antibiotics Chloramphenicol, penicillins, sulfonamides, p-aminosalicylic acid (PAS), rifampin, vancomycin, isoniazid, nitrofurantoin Antimalarials Dapsone, quinine, pyrimethamine Anticonvulsants Phenytoin, mephenytoin, trimethadione, ethosuximide, carbamazepine Hypoglycemic agents Tolbutamide, chlorpropamide Antihistamines Cimetidine, brompheniramine, tripelennamine Antihypertensives Methyldopa, captopril Antiinflammatory agents Aminopyrine, phenylbutazone, gold salts, ibuprofen, indomethacin Antithyroid agents Propylthiouracil, methimazole, thiouracil Diuretics Acetazolamide, hydrochlorothiazide, chlorthalidone Phenothiazines Chlorpromazine, promazine, prochlorperazine Immunosuppressive agents Antimetabolites Cytotoxic agents Alkylating agents, antimetabolites, anthracyclines, Vinca alkaloids, cisplatin, hydroxyurea, dactinomycin Other agents Recombinant interferons, allopurinol, ethanol, levamisole, penicillamine, zidovudine, streptokinase, carbamazepine, clopidogrel, ticlopidine Modified from Goldman L, Ausiello D (eds): Cecil textbook of medicine, ed 22, Philadelphia, 2004, WB Saunders.

NOREPINEPHRINE Normal range: 0-600 pg/ml Elevated in: Pheochromocytomas, stress, vigorous exercise, certain foods (bananas, chocolate, coffee, tea, vanilla)

5-NUCLEOTIDASE Normal range: 2-16 IU/L Elevated in: Biliary obstruction, metastatic neoplasms to liver, primary biliary cirrhosis, renal failure, pancreatic carcinoma, chronic active hepatitis

OSMOLALITY (serum) Normal range: 280-300 mOsm/kg It can also be estimated by the following formula: 2([Na]  [K])  glucose/18 BUN/2.8 Elevated in: Dehydration, hypernatremia, diabetes insipidus, uremia, hyperglycemia, mannitol therapy, ingestion of toxins (ethylene glycol, methanol, ethanol), hypercalcemia, diuretics Decreased in: Syndrome of inappropriate diuretic hormone secretion, hyponatremia, overhydration, Addison’s disease, hypothyroidism

PARACENTESIS FLUID Testing and evaluation of results: 1. Process the fluid as follows: a. Tube 1: LDH, glucose, albumin. b. Tube 2: protein, specific gravity. c. Tube 3: cell count and differential. d. Tube 4: save until further notice. 2. Draw serum LDH, protein, albumin.

3. Gram stain, AFB stain, bacterial and fungal cultures, amylase, and triglycerides should be ordered only when clearly indicated; bedside inoculation of blood-culture bottles with ascitic fluid improves sensitivity in detecting bacterial growth. 4. If malignant ascites is suspected, consider a carcinoembryonic antigen level on the paracentesis fluid and cytologic evaluation. 5. In suspected spontaneous bacterial peritonitis (SBP) the incidence of positive cultures can be increased by injecting 10 to 20 ml of ascitic fluid into blood culture bottles. 6. Peritoneal effusion can be subdivided as exudative or transudative based on its characteristics. 7. The serum-ascites albumin gradient (serum albumin level-ascitic fluid albumin level) correlates directly with portal pressure and can also be used to classify ascite. Patients with gradients 1.1 g/dl have portal hypertension, and those with gradients 1.1 g/dl do not; the accuracy of this method is 95%. 8. An ascitic fluid polymorphonuclear leukocyte count 500/l is suggestive of SBP. 9. A blood-ascitic fluid albumin gradient.

PARTIAL THROMBOPLASTIN TIME (PTT), ACTIVATED PARTIAL THROMBOPLASTIN TIME (APTT) Normal range: 25-41 sec Elevated in: Heparin therapy, coagulation factor deficiency (I, II, V, VIII, IX, X, XI, XII), liver disease, vitamin K deficiency, disseminated intravascular coagulation, circulating anticoagulant, warfarin therapy, specific factor inhibition (PCN reaction, rheumatoid arthritis), thrombolytic therapy, nephrotic syndrome NOTE: Useful to evaluate the intrinsic coagulation system.

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PH, Blood PH, BLOOD Normal values: Arterial: 7.35-7.45 Venous: 7.32-7.42 For abnormal values refer to “Arterial Blood Gases.”

PHOSPHATASE, ACID; see ACID PHOSPHATASE PHOSPHATASE, ALKALINE; see ALKALINE PHOSPHATASE PHOSPHATE (serum) Normal range: 2.5-5 mg/dl DECREASED Parenteral hyperalimentation Diabetic acidosis Alcohol withdrawal Severe metabolic or respiratory alkalosis Antacids that bind phosphorus Malnutrition with refeeding using low-phosphorus nutrients Renal tubule failure to reabsorb phosphate (Fanconi’s syndrome; congenital disorder; vitamin D deficiency) Glucose administration Nasogastric suction Malabsorption Gram-negative sepsis Primary hyperparathyroidism Chlorothiazide diuretics Therapy of acute severe asthma Acute respiratory failure with mechanical ventilation INCREASED Renal failure Severe muscle injury Phosphate-containing antacids Hypoparathyroidism Tumor lysis syndrome

PLATELET COUNT

Potassium

435

e. Autoimmune hemolytic anemia f. Lymphoreticular disorders (e.g., chronic lymphocytic leukemia) 2. Nonimmunologic a. Prosthetic heart valves b. Thrombotic thrombocytopenic purpura c. Sepsis d. Disseminated intravascular coagulation e. Hemolytic-uremic syndrome f. Giant cavernous hemangioma B. Decreased production 1. Abnormal marrow a. Marrow infiltration (e.g., leukemia, lymphoma, fibrosis) b. Marrow suppression (e.g., chemotherapy, alcohol, radiation) 2. Hereditary disorders a. Wiskott-Aldrich syndrome: X-linked disorder characterized by thrombocytopenia, eczema, and repeated infections b. May-Hegglin anomaly: increased megakaryocytes but ineffective thrombopoiesis 3. Vitamin deficiencies (e.g., vitamin B12, folic acid) C. Splenic sequestration, hypersplenism D. Dilutional, secondary to massive transfusion

POTASSIUM (serum)

LABORATORY TESTS AND INTERPRETATION

Normal range: 3.5-5 mEq/L CAUSES OF HYPERKALEMIA I. Pseudohyperkalemia A. Hemolysis of sample B. Thrombocytosis C. Leukocytosis D. Laboratory error II. Increased potassium intake and absorption A. Potassium supplements (oral and parenteral) B. Dietary—salt substitutes C. Stored blood D. Potassium-containing medications III. Impaired renal excretion A. Acute renal failure B. Chronic renal failure C. Tubular defect in potassium secretion 1. Renal allograft 2. Analgesic nephropathy 3. Sickle cell disease 4. Obstructive uropathy 5. Interstitial nephritis 6. Chronic pyelonephritis 7. Potassium-sparing diuretics 8. Miscellaneous (lead, systemic lupus erythematosus, pseudohypoaldosteronism) D. Hypoaldosteronism 1. Primary (Addison’s disease) 2. Secondary a. Hyporeninemic hypoaldosteronism (type IV RTA) b. Drug-induced (1) Nonsteroidal antiinflammatory medications (2) ACE inhibitors (3) Heparin (4) Cyclosporine IV. Transcellular shifts A. Acidosis B. Hypertonicity C. Insulin deficiency D. Drugs 1. -blockers 2. Digitalis toxicity 3. Succinylcholine

Section IV

Normal range: 130-400 103/mm3 Elevated in: REACTIVE THROMBOCYTOSIS Infections or inflammatory states—vasculitis, allergic reactions, etc. Surgery and tissue damage—myocardial infarction, pancreatitis, etc. Postsplenectomy state Malignancy—solid tumors, lymphoma Iron deficiency anemia, hemolytic anemia, acute blood loss Uncertain etiology Rebound effect after chemotherapy or immune thrombocytopenia Renal disorders—renal failure, nephrotic syndrome MYELOPROLIFERATIVE DISORDERS Chronic myeloid leukemia Primary thrombocythemia Polycythemia vera Idiopathic myelofibrosis Decreased: A. Increased destruction 1. Immunologic a. Drugs: quinine, quinidine, digitalis, procainamide, thiazide diuretics, sulfonamides, phenytoin, aspirin, penicillin, heparin, gold, meprobamate, sulfa drugs, phenylbutazone, NSAIDs, methyldopa, cimetidine, furosemide, INH, cephalosporins, chlorpropamide, organic arsenicals, chloroquine b. Idiopathic thrombocytopenic purpura c. Transfusion reaction: transfusion of platelets with platelet antigen HPA-1a (PLA1) in recipients without PLA1 d. Vasculitis (e.g., systemic lupus erythematosus)

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Prostate-Specific Antigen E. Exercise F. Hyperkalemic periodic paralysis V. Cellular injury A. Rhabdomyolysis B. Severe intravascular hemolysis C. Acute tumor lysis syndrome D. Burns and crush injuries CAUSES OF HYPOKALEMIA I. Decreased intake A. Decreased dietary potassium B. Impaired absorption of potassium C. Clay ingestion D. Kayexalate II. Increased loss A. Renal 1. Hyperaldosteronism a. Primary 1. Conn’s syndrome 2. Adrenal hyperplasia b. Secondary 1. Congestive heart failure 2. Cirrhosis 3. Nephrotic syndrome 4. Dehydration c. Bartter’s syndrome 2. Glycyrrhizic acid (licorice, chewing tobacco) 3. Excessive adrenal corticosteroids a. Cushing’s syndrome b. Steroid therapy c. Adrenogenital syndrome 4. Renal tubular defects a. Renal tubular acidosis b. Obstructive uropathy c. Salt-wasting nephropathy 5. Drugs a. Diuretics b. Aminoglycosides c. Mannitol d. Amphotericin e. Cisplatin f. Carbenicillin B. Gastrointestinal 1. Vomiting 2. Nasogastric suction 3. Diarrhea 4. Malabsorption 5. Ileostomy 6. Villous adenoma 7. Laxative abuse C. Increased losses from the skin 1. Excessive sweating 2. Burns III. Transcellular shifts A. Alkalosis 1. Vomiting 2. Diuretics 3. Hyperventilation 4. Bicarbonate therapy B. Insulin 1. Exogenous 2. Endogenous response to glucose C. 2-Agonists (albuterol, terbutaline, epinephrine) D. Hypokalemia periodic paralysis 1. Familial 2. Thyrotoxic IV. Miscellaneous

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Prostate-Specific Antigen A. Anabolic state B. Intravenous hyperalimentation C. Treatment of megaloblastic anemia D. Acute mountain sickness

PROSTATE-SPECIFIC ANTIGEN (PSA) Normal range: 0-4 ng/ml Table 4-16 describes age-specific reference ranges for PSA. Elevated in: Benign prostatic hypertrophy, carcinoma of prostate, postrectal examination, prostate trauma Factors affecting serum PSA are described in Table 4-17. NOTE: Measurement of free PSA is useful to assess the probability of prostate cancer in patients with normal digital rectal examination and total PSA between 4 and 10 ng/ml. In these patients, the global risk of prostate cancer is 25%; however, if the free PSA is 25%, the risk of prostate cancer decreases to 8%, whereas if the free PSA is 10%, the risk of cancer increases to 56%. Free PSA is also useful to evaluate the aggressiveness of prostate cancer. A low free PSA percentage generally indicates a high-grade cancer, whereas a high free PSA percentage is generally associated with a slower growing tumor or BPH. Decreased in: Finasteride therapy, dutasteride therapy, saw palmetto use, bedrest, antiandrogens

TABLE 4-16

Age-Specific Reference Ranges

for PSA SERUM PSA (NG/ML)

Age (yr)

Whites

Japanese

African American

40-49 50-59 60-69 70-79

0-2.5 0-3.5 0-4.5 0-6.5

0-2.0 0-3.0 0-4.0 0-5.0

0-2.0 0-4.0 0-4.5 0-5.5

From Nseyo UO (ed): Urology for primary care physicians, Philadelphia, 1999, WB Saunders. PSA, Prostate-specific antigen.

TABLE 4-17 Factors Affecting Serum ProstateSpecific Antigen (PSA) Factors Affecting Serum PSA

Duration of Effect

Prostate size Recent ejaculation Prostate manipulation Vigorous massage Cystoscopy Prostate biopsy Prostatitis Acute Chronic Prostate cancer Drugs: finasteride (Proscar)*

Not applicable 6-48 hours 1 week 1 week 4-6 weeks 3-6 months Unknown Not applicable 3-6 months

From Nseyo UO (ed): Urology for primary care physicians, Philadelphia, 1999, WB Saunders. *Lowers PSA for as long as patient is on the medication.

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Protein PROTEIN (serum) Normal range: 6-8 g/dl Elevated in: Dehydration, multiple myeloma, Waldenström’s macroglobulinemia, sarcoidosis, collagen-vascular diseases Decreased in: Malnutrition, low-protein diet, overhydration, malabsorption, severe burns, neoplasms, chronic diseases, cirrhosis, nephrosis

PROTEIN ELECTROPHORESIS (serum) Normal range: Albumin: 60% to 75%

-1: 1.7% to 5%

-2: 6.7% to 12.5% : 8.3% to 16.3% : 10.7% to 20% Albumin: 3.6-5.2 g/dl

-1: 0.1-0.4 g/dl

-2: 0.4-1 g/dl

Protein Electrophoresis

437

: 0.5-1.2 g/dl : 0.6-1.6 g/dl Elevated in: Albumin: dehydration

-1: neoplastic diseases, inflammation

-2: neoplasms, inflammation, infection, nephrotic syndrome : hypothyroidism, biliary cirrhosis, diabetes mellitus : see IMMUNOGLOBULINS Decreased in: Albumin: malnutrition, chronic liver disease, malabsorption, nephrotic syndrome, burns, systemic lupus erythematosus

-1: emphysema ( -1 antitrypsin deficiency), nephrosis

-2: hemolytic anemias (decreased haptoglobin), severe hepatocellular damage : hypocholesterolemia, nephrosis : see IMMUNOGLOBULINS Fig. 4-10 describes serum protein electrophoretic patterns.

Section IV LABORATORY TESTS AND INTERPRETATION

FIGURE 4-10 Typical serum protein electrophoretic patterns. 1, Normal (arrow near  region indicates serum application point). 2, Acute reaction pattern. 3, Acute reaction or nephrotic syndrome. 4, Nephrotic syndrome. 5, Chronic inflammation, cirrhosis, granulomatous diseases, rheumatoid-collagen group. 6, Same as 5, but g elevation is more pronounced. There is also partial (but not complete) - fusion. 7, Suggestive of cirrhosis but could be found in the granulomatous diseases or the rheumatoidcollagen group. 8, Characteristic pattern of cirrhosis. 9, -1 Antitrypsin deficiency with mild  elevation suggesting concurrent chronic disease. 10, Same as 5, but the  elevation is marked. The configuration of the  peak superficially mimics that of myeloma, but is more broad-based. There are superimposed acute reaction changes. 11, Hypogammaglobulinemia or light-chain myeloma. 12, Myeloma, Waldenström’s macroglobulinemia, idiopathic or secondary monoclonal gammopathy. (From Ravel R: Clinical laboratory medicine, ed 6, St Louis, 1995, Mosby.)

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Prothrombin Time

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Sodium

PROTHROMBIN TIME (PT)

RHEUMATOID FACTOR

Normal range: 10-12 sec Elevated in: Liver disease, oral anticoagulants (warfarin), heparin, factor deficiency (I, II, V, VII, X), disseminated intravascular coagulation, vitamin K deficiency, afibrinogenemia, dysfibrinogenemia, drugs (salicylate, chloral hydrate, diphenylhydantoin, estrogens, antacids, phenylbutazone, quinidine, antibiotics, allopurinol, anabolic steroids) Decreased in: Vitamin K supplementation, thrombophlebitis, drugs (glutethimide, estrogens, griseofulvin, diphenhydramine)

Normal: Negative Present in titer 1:20 RHEUMATIC DISEASES Rheumatoid arthritis Sjögren’s syndrome Systemic lupus erythematosus Polymyositis/dermatomyositis Mixed connective tissue disease Scleroderma INFECTIOUS DISEASES Subacute bacterial endocarditis Tuberculosis Infectious mononucleosis Hepatitis Syphilis Leprosy Influenza MALIGNANCIES Lymphoma Multiple myeloma Waldenström’s macroglobulinemia Postradiation or postchemotherapy MISCELLANEOUS Normal adults, especially the elderly Sarcoidosis Chronic pulmonary disease (interstitial fibrosis) Chronic liver disease (chronic active hepatitis, cirrhosis) Mixed essential cryoglobulinemia Hypergammaglobulinemic purpura

PROTOPORPHYRIN (free erythrocyte) Normal range: 16-36 g/dl of red blood cells Elevated in: Iron deficiency, lead poisoning, sideroblastic anemias, anemia of chronic disease, hemolytic anemias, erythropoietic protoporphyria

PSA; see PROSTATE-SPECIFIC ANTIGEN PT; see PROTHROMBIN TIME PTT; see PARTIAL THROMBOPLASTIN TIME RDW; see RED BLOOD CELL DISTRIBUTION WIDTH RED BLOOD CELL (RBC) COUNT Normal range: Male: 4.3-5.9 106/mm3 Female: 3.5-5 106/mm3 Elevated in: Polycythemia vera, smokers, high altitude, cardiovascular disease, renal cell carcinoma and other erythropoietinproducing neoplasms, stress, hemoconcentration/dehydration Decreased in: Anemias, hemolysis, chronic renal failure, hemorrhage, failure of marrow production

RED BLOOD CELL DISTRIBUTION WIDTH (RDW) Measures variability of red cell size (anisocytosis) Normal range: 11.5-14.5 Normal RDW and: ELEVATED MEAN CORPUSCULAR VOLUME (MCV): aplastic anemia, preleukemia NORMAL MCV: normal, anemia of chronic disease, acute blood loss or hemolysis, chronic lymphocytic leukemia (CLL), chronic myelocytic leukemia, nonanemic enzymopathy or hemoglobinopathy DECREASED MCV: anemia of chronic disease, heterozygous thalassemia Elevated RDW and: ELEVATED MCV: vitamin B12 deficiency, folate deficiency, immune hemolytic anemia, cold agglutinins, CLL with high count, liver disease NORMAL MCV: early iron deficiency, early vitamin B12 deficiency, early folate deficiency, anemic globinopathy DECREASED MCV: iron deficiency, red blood cell fragmentation, HbH disease, thalassemia intermedia

RED BLOOD CELL FOLATE; see FOLATE, RED BLOOD CELL RETICULOCYTE COUNT Normal range: 0.5% to 1.5% Elevated in: Hemolytic anemia (sickle cell crisis, thalassemia major, autoimmune hemolysis), hemorrhage, postanemia therapy (folic acid, ferrous sulfate, vitamin B12), chronic renal failure Decreased in: Aplastic anemia, marrow suppression (sepsis, chemotherapeutic agents, radiation), hepatic cirrhosis, blood transfusion, anemias of disordered maturation (iron deficiency anemia, megaloblastic anemia, sideroblastic anemia, anemia of chronic disease)

RNP; see EXTRACTABLE NUCLEAR ANTIGEN SEDIMENTATION RATE; see ERYTHROCYTE SEDIMENTATION RATE SGOT; see ASPARTATE AMINOTRANSFERASE SGPT; see ALANINE AMINOTRANSFERASE SMOOTH MUSCLE ANTIBODY Normal: Negative Present in: Chronic acute hepatitis (1:80), primary biliary cirrhosis (1:80), infectious mononucleosis

SODIUM (serum) Normal range: 135-147 mEq/L HYPONATREMIA A. Sodium and water depletion (deficit hyponatremia) 1. Loss of gastrointestinal secretions with replacement of fluid but not electrolytes a. Vomiting b. Diarrhea c. Tube drainage 2. Loss from skin with replacement of fluids but not electrolytes a. Excessive sweating b. Extensive burns 3. Loss from kidney a. Diuretics b. Chronic renal insufficiency (uremia) with acidosis 4. Metabolic loss a. Starvation with acidosis b. Diabetic acidosis 5. Endocrine loss a. Addison’s disease b. Sudden withdrawal of long-term steroid therapy 6. Iatrogenic loss from serous cavities a. Paracentesis or thoracentesis

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Synovial Fluid Analysis B. Excessive water (dilution hyponatremia) 1. Excessive water administration 2. Congestive heart failure 3. Cirrhosis 4. Nephrotic syndrome 5. Hypoalbuminemia (severe) 6. Acute renal failure with oliguria C. Inappropriate antidiuretic hormone (IADH) syndrome D. Intracellular loss (reset osmostat syndrome) E. False hyponatremia (actually a dilutional effect) 1. Marked hypertriglyceridemia* 2. Marked hyperproteinemia* 3. Severe hyperglycemia HYPERNATREMIA Dehydration is the most frequent overall clinical finding in hypernatremia. 1. Deficient water intake (either orally or intravenously) 2. Excess kidney water output (diabetes insipidus, osmotic diuresis) 3. Excess skin water output (excess sweating, loss from burns) 4. Excess gastrointestinal tract output (severe protracted vomiting or diarrhea without fluid therapy) 5. Accidental sodium overdose 6. High-protein tube feedings

SYNOVIAL FLUID ANALYSIS Table 4-18 describes the classification and interpretation of synovial fluid analysis.

T3 (triiodothyronine) Normal range: 75-220 ng/dl Abnormal values: A. Elevated in hyperthyroidism (usually earlier and to a greater extent than serum T4). B. Useful in diagnosing: 1. T3 hyperthyroidism (thyrotoxicosis): increased T3, normal FTI.

TABLE 4-18

T4, Serum T4, and Free 2. Toxic nodular goiter: increased T3, normal or increased T 4. 3. Iodine deficiency: normal T3, possibly decreased T4. 4. Thyroid replacement therapy with liothyronine (Cytomel): normal T4, increased T3 if patient is symptomatically hyperthyroid. Not ordered routinely but indicated when hyperthyroidism is suspected and serum free T4 or FTI inconclusive.

T3 (triiodothyronine); see Table 4-19 for T3 abnormalities T3 RESIN UPTAKE (T3RU) Normal range: 25% to 35% Abnormal values: Increased in hyperthyroidism. T3 resin uptake (T3RU or RT3U) measures the percentage of free T4 (not bound to protein); it does not measure serum T3 concentration; T3RU and other tests that reflect thyroid hormone binding to plasma protein are also known as thyroid hormone-binding ratios (THBR).

T4, SERUM T4, AND FREE (free thyroxine) Normal range: 0.8-2.8 ng/dl Abnormal values: Serum thyroxine (T4) Elevated in: 1. Graves’ disease 2. Toxic multinodular goiter 3. Toxic adenoma 4. Iatrogenic and factitious 5. Transient hyperthyroidism. a. Subacute thyroiditis b. Hashimoto’s thyroiditis c. Silent thyroiditis 6. Rare causes: hypersecretion of TSH (e.g., pituitary neoplasms), struma ovarii, ingestion of large amounts of iodine in a patient with preexisting thyroid hyperplasia or adenoma

Classification and Interpretation of Synovial Fluid Analysis

Protein (g/dl)

— Osteoarthritis, aseptic necrosis, traumatic arthritis, erythema nodosum, osteochondritis dissecans Crystal-induced arthritis, rheumatoid arthritis, Reiter’s syndrome, collagenvascular disease, psoriatic arthritis, serum sickness, rheumatic fever Bacterial (staphylococcal, gonococcal, tuberculosis)

Clear Clear, yellow (may be xanthochromic if traumatic arthritis)

↑ ↑

Firm Firm

200 ↑ Up to 10,000

25 25

10 10

2.5 2.5

Clear, yellow, turbid

↓

Friable

↑↑ Up to 100,000

40-90

40

.2.5

↓/↑

Friable

↑↑↑ Up to 5 million

40-100

20-100

.2.5

↑, Elevated; ↑↑, markedly high; ↓, decreased; PMN, polymorphonuclear leukocytes. Note that there is considerable overlap in the numbers listed above.

LABORATORY TESTS AND INTERPRETATION

Normal I (noninflammatory)

Turbid

WBC/MM3

%PMN

Appearance

Section IV

Mucin Clot

Diseases

III (septic)

Viscosity

Glucose (mg/dl) (Blood– Synovial Fluid)

Group

II (inflammatory)

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T4, Free

TABLE 4-19

Thoracentesis Fluid

Findings in Thyroid Function Tests in Various Clinical Conditions

Condition Hyperthyroidism Graves’ disease Toxic nodular goiter Pituitary TSH-secreting tumors T3 thyrotoxicosis T4 thyrotoxicosis Hypothyroidism Primary Secondary Tertiary Peripheral unresponsiveness

T4

FT4I

T3

FT3I

TSH

TSI

TRH Stimulation

↑ ↑ ↑ N ↑

↑ ↑ ↑ N ↑

↑ ↑ ↑ ↑ N

↑ ↑ ↑ ↑ N

↓ ↓ ↑ ↓ ↓

   ,  , 

↓ ↓ ↓ ↓ ↓

↓ ↓ ↓ ↑, N

↓ ↓ ↓ ↑, N

↓ ↓ ↓ ↑, N

↓ ↓ ↓ ↑

↑ ↓, N ↓, N ↑, N

,    

↑ ↓ N N, ↑

From Tilton RC, Barrows A: Clinical laboratory medicine, St Louis, 1992, Mosby. N, Normal; ↑, increased; ↓, decreased; ,  variable.

(Jod-Basedow phenomenon), carcinoma of thyroid, amiodarone therapy of arrhythmias. Serum thyroxine test measures both circulating thyroxine bound to protein (represents 99% of circulating T4) and unbound (free) thyroxine. Values vary with protein binding; changes in the concentration of T4 secondary to changes in thyroxine-binding globulin (TBG) can be caused by the following: Increased TBG (↑T4) Estrogens Acute infectious hepatitis Familial Fluorouracil, clofibrate, heroin, methadone

Decreased TBG (↓T4) Androgens, glucocorticoids Nephrotic syndrome, cirrhosis Acromegaly Hypoproteinemia Familial Phenytoin, ASA and other NSAIDs, high-dose penicillin, asparaginase Chronic debilitating illness To eliminate the suspected influence of protein binding on thyroxine values, two additional tests are available: T3 resin uptake and serum free thyroxine.

T4, FREE (free thyroxine) Normal range: 0.8-2.8 ng/dl Elevated in: Graves’ disease, toxic multinodular goiter, toxic adenoma, iatrogenic and factitious causes, transient hyperthyroidism Serum free T4 directly measures unbound thyroxine. Free T4 can be measured by equilibrium dialysis (gold standard of free T4 assays) or by immunometric techniques (influenced by serum levels of lipids, proteins, and certain drugs). The free thyroxine index (FTI) can also be easily calculated by multiplying T4 times T3RU and dividing the result by 100; the FTI corrects for any abnormal T4 values secondary to protein binding: FTI T4 T3RU/100. Normal values equal 1.1 to 4.3.

TESTOSTERONE (total testosterone) Normal range: (Variable with age and sex) Serum/plasma: Males: 280-1100 ng/dl Females: 15-70 ng/dl Urine: Males: 50-135 g/day Females: 2-12 g/day Elevated in: Testicular tumors, ovarian masculinizing tumors Decreased in: Hypogonadism

THORACENTESIS FLUID Testing and evaluation of results: 1. Pleural effusion fluid should be differentiated in exudate or transudate. The initial laboratory studies should be aimed only at distinguishing an exudate from a transudate. a. Tube 1: protein, LDH, albumin. b. Tubes 2, 3, 4: save the fluid until further notice. In selected patients with suspected empyema, a pH level may be useful (generally 7.0). See following for proper procedure to obtain a pH level from pleural fluid. NOTE: Do not order further tests until the presence of an exudate is confirmed on the basis of protein and LDH determinations (Section III, Fig. 3-70); however, if the results of protein and LDH determinations cannot be obtained within a reasonable time (resulting in unnecessary delay), additional laboratory tests should be ordered at the time of thoracentesis. 2. A serum/effusion albumin gradient of 1.2 g/dl is indicative of exudative effusions, especially in patients with congestive heart failure (CHF) treated with diuretics. 3. Note the appearance of the fluid: a. A grossly hemorrhagic effusion can be a result of a traumatic tap, neoplasm, or an embolus with infarction. b. A milky appearance indicates either of the following: (1) Chylous effusion: caused by trauma or tumor invasion of the thoracic duct; lipoprotein electrophoresis of the effusion reveals chylomicrons and triglyceride levels 115 mg/dl. (2) Pseudochylous effusion: often seen with chronic inflammation of the pleural space (e.g., TB, connective tissue diseases). 4. If transudate, consider CHF, cirrhosis, chronic renal failure, and other hypoproteinemic states and perform subsequent workup accordingly. 5. If exudate, consider ordering these tests on the pleural fluid: a. Cytologic examination for malignant cells (for suspected neoplasm). b. Gram stain, cultures (aerobic and anaerobic), and sensitivities (for suspected infectious process). c. AFB stain and cultures (for suspected TB). d. pH: a value  7.0 suggests parapneumonic effusion or empyema; a pleural fluid pH must be drawn anaerobically and iced immediately; the syringe should be prerinsed with 0.2 ml of 1:1000 heparin. e. Glucose: a low glucose level suggests parapneumonic effusions and rheumatoid arthritis.

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Thyroid-Stimulating Hormone f. Amylase: a high amylase level suggests pancreatitis or ruptured esophagus. g. Perplexing pleural effusions are often a result of malignancy (e.g., lymphoma, malignant mesothelioma, ovarian carcinoma), TB, subdiaphragmatic processes, prior asbestos exposure, and postcardiac injury syndrome.

THYROID-STIMULATING HORMONE (TSH)

Normal range: 4-11 g/dl

TIBC; see IRON-BINDING CAPACITY TRANSFERRIN Normal range: 170-370 mg/dl Elevated in: Iron deficiency anemia, viral hepatitis

441

Decreased in: Nephrotic syndrome, liver disease, hereditary deficiency, protein malnutrition, neoplasms, chronic inflammatory states, chronic illness, thalassemia, hemochromatosis, hemolytic anemia

TRIGLYCERIDES Normal range: 150 mg/dl Elevated in: Hyperlipoproteinemias (types I, IIb, III, IV, V), hypothyroidism, estrogens, acute myocardial infarction, pancreatitis, alcohol intake, nephrotic syndrome, diabetes mellitus, glycogen storage disease Decreased in: Malnutrition, congenital abetalipoproteinemias, drugs (e.g., gemfibrozil, fenofibrate, nicotinic acid, clofibrate)

TRIIODOTHYRONINE; see T3 TROPONINS, SERUM Normal range: 0-0.4 ng/ml (negative). If there is clinical suspicion of evolving acute MI or ischemic episode, repeat testing in 5-6 hours is recommended. Indeterminate: 0.05-0.49 ng/ml. Suggest further tests. In a patient with unstable angina and this troponin I level, there is an increased risk of a cardiac event in the near future. Strong probability of acute MI: 0.05 ng/ml CARDIAC TROPONIN T (CTNT) is a highly sensitive marker for myocardial injury for the first 48 hours after MI and for up to 5-7 days (see Fig. 4-2, under “Creatine Kinase Isoenzymes”). It may be also elevated in renal failure, chronic muscle disease, and trauma. CARDIAC TROPONIN I (CTNI) is highly sensitive and specific for myocardial injury (CK-MB) in the initial 8 hours, peaks within 24 hours, and lasts up to 7 days. With progressively higher levels of cTnI, the risk of mortality increases because the amount of necrosis increases.

TSH; see THYROID-STIMULATING HORMONE TT; see THROMBIN TIME TUBERCULIN TEST (PPD) Abnormal results: see Boxes 4-3 and 4-4 for interpretation

UNCONJUGATED BILIRUBIN; see BILIRUBIN, INDIRECT UREA NITROGEN, BLOOD (BUN) Normal range: 8-18 mg/dl Box 4-5 describes factors affecting BUN level independent of renal function. Elevated in: Drugs (aminoglycosides and other antibiotics, diuretics, lithium, corticosteroids), dehydration, gastrointestinal bleeding, decreased renal blood flow (shock, congestive heart failure, myocardial infarction), renal disease (glomerulonephritis, pyelonephritis, diabetic nephropathy), urinary tract obstruction (prostatic hypertrophy) Decreased in: Liver disease, malnutrition, overhydration, acromegaly, celiac disease

URIC ACID (serum) Normal range: 2-7 mg/dl Elevated in: Renal failure, gout, excessive cell lysis (chemotherapeutic agents, radiation therapy, leukemia, lymphoma, hemolytic anemia), hereditary enzyme deficiency (hypoxanthine-guaninephosphoribosyl transferase), acidosis, myeloproliferative disorders, diet high in purines or protein, drugs (diuretics, low doses of ASA, ethambutol, nicotinic acid), lead poisoning, hypothyroidism, Addison’s disease, nephrogenic diabetes insipidus, active psoriasis, polycystic kidneys Decreased in: Drugs (allopurinol, high doses of ASA, probenecid, warfarin, corticosteroid), deficiency of xanthine

LABORATORY TESTS AND INTERPRETATION

THYROXINE (T4)

Uric Acid

Section IV

Normal range: 2-11 U/ml CONDITIONS THAT INCREASE SERUM THYROID-STIMULATING HORMONE VALUES Laboratory error Primary hypothyroidism Synthroid therapy with insufficient dose Lithium or amiodarone; some patients Hashimoto’s thyroiditis in later stage Large doses of inorganic iodide (e.g., SSKI) Severe nonthyroid illness in recovery phase Iodine deficiency (moderate or severe) Addison’s disease TSH specimen drawn in evening (peak of diurnal variation) Pituitary TSH-secreting tumor Therapy of hypothyroidism (3-6 wk after beginning therapy [range, 1-8 wk]; sometimes longer when pretherapy TSH is over 100 U/ml) Acute psychiatric illness Peripheral resistance to T4 syndrome Antibodies (e.g., HAMA) interfering with monoclonal sandwich method of TSH assay Telepaque (iopanoic acid) and Oragrafin (ipodate) x-ray contrast media Amphetamines High altitudes CONDITIONS THAT DECREASE SERUM THYROID-STIMULATING HORMONE VALUES Laboratory error T4/T3 toxicosis (diffuse or nodular etiology) Excessive therapy for hypothyroidism Active thyroiditis (subacute, painless, or early active Hashimoto’s disease) Multinodular goiter containing areas of autonomy Severe nonthyroid illness (especially acute trauma, dopamine, or glucocorticoid) T3 toxicosis Pituitary insufficiency Cushing’s syndrome (and some patients on high-dose glucocorticoid) Jod-Basedow (iodine-induced) hyperthyroidism Thyroid-stimulating hormone drawn 2-4 hr after levothyroxine dose Postpartum transient toxicosis Factitious hyperthyroidism Struma ovarii Radioimmunoassay, surgery, or antithyroid drug therapy for hyperthyroidism 4-6 wk (range 2 wk–2 yr) after the treatment Interleukin-2 drugs (3%-6% of cases) or -interferon therapy (1% of cases) Hyperemesis gravidarum Amiodarone therapy

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Urinalysis

BOX 4-3

VDRL

PPD Reaction Size Considered “Positive” (Intracutaneous 5 TU Mantoux Test at 48 hr)

5 mm or More HIV infection or risk factors for HIV Close recent contact with active TB case Persons with chest x-ray consistent with healed TB 10 mm or More Foreign-born persons from countries with high TB prevalence in Asia, Africa, and Latin America IV drug users Medically underserved low-income population groups (including Native Americans, Hispanics, and blacks)

Residents of long-term care facilities (nursing homes, mental institutions) Medical conditions that increase risk for TB (silicosis, gastrectomy, undernourished, diabetes mellitus, high-dose corticosteroids or immunosuppression Rx, leukemia or lymphoma, other malignancies) Employees of long-term care facilities, schools, child-care facilities, health care facilities 15 mm or More All others not already listed

TB, Tuberculosis; TU, tuberculin units.

BOX 4-4

Factors Associated with False-Negative Tuberculin Tests

Technical Errors Improper administration Inaccurate reading Loss of potency of antigen Patient-Related Factors (Anergy) Age (elderly) Nutritional status

Medications—corticosteroids, immunosuppressive agents Severe tuberculosis Coexisting diseases HIV infection Viral illness or vaccination Lymphoreticular malignancies Sarcoidosis Solid tumors

Lepromatous leprosy Sjögren’s syndrome Ataxia telangiectasia Uremia Primary biliary cirrhosis Systemic lupus erythematosus Severe systemic disease of any etiology

From Stein JH (ed): Internal medicine, ed 4, St Louis, 1994, Mosby.

BOX 4-5

Factors Affecting Blood Urea Nitrogen Level Independent of Renal Function

Disproportionate Increase in Blood Urea Nitrogen Volume depletion “prerenal azotemia” Gastrointestinal hemorrhage Corticosteroid or cytotoxic agents High-protein diet Obstructive uropathy

Sepsis Catabolic states tissue breakdown Disproportionate Decrease in Blood Urea Nitrogen Low-protein diet Liver disease

From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders.

oxidase, syndrome of inappropriate antidiuretic hormone secretion, renal tubular deficits (Fanconi’s syndrome), alcoholism, liver disease, diet deficient in protein or purines, Wilson’s disease, hemochromatosis

URINALYSIS Normal range: Color: light straw Appearance: clear Ketones: absent pH: 4.5-8 (average, 6) Protein: absent Glucose: absent Specific gravity: 1.005-1.030 Occult blood absent Microscopic examination: Red blood cells: 0-5 (high-power field) White blood cells: 0-5 (high-power field) Bacteria (spun specimen): absent Casts: 0-4 hyaline (low-power field) Abnormalities in the microscopic examination of urine are described in Table 4-20.

URINE 5-HYDROXYINDOLE-ACETIC ACID (urine 5HIAA) Normal range: 2-8 mg/24 hr

Elevated in: Carcinoid tumors, after ingestion of certain foods (bananas, plums, tomatoes, avocados, pineapples, eggplant, walnuts), drugs (monoamine oxidase inhibitors, phenacetin, methyldopa, glycerol guaiacolate, acetaminophen, salicylates, phenothiazines, imipramine, methocarbamol, reserpine, methamphetamine)

URINE VANILLYLMANDELIC ACID (VMA) Normal range: 6.8 mg/24 hr Elevated in: Pheochromocytoma, drugs (isoproterenol, methocarbamol, levodopa, sulfonamides, chlorpromazine), severe stress, after ingestion of bananas, chocolate, vanilla, tea, coffee Decreased in: Drugs (monoamine oxidase inhibitors, reserpine, guanethidine, methyldopa)

VDRL Normal range: Negative Positive test: Syphilis, other treponemal diseases (yaws, pinta, bejel) NOTE: A false-positive test may be seen in patients with systemic lupus erythematosus and other autoimmune diseases, infectious mononucleosis, HIV, atypical pneumonia, malaria, leprosy, typhus fever, rat-bite fever, relapsing fever. NOTE: See Table 4-21 for interpretation of serologic tests for syphilis.

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●

VDRL

TABLE 4-20

VDRL

443

Microscopic Examination of the Urine

Finding

Associations

Casts Red blood cell White blood cell Epithelial cell Granular Waxy, broad Hyaline Fatty Cells Red blood cell White blood cell Eosinophil (Squamous) epithelial cell Crystals Uric acid Calcium phosphate Calcium oxalate Cystine Sulfur

Glomerulonephritis, vasculitis Interstitial nephritis, pyelonephritis Acute tubular necrosis, interstitial nephritis, glomerulonephritis Renal parenchymal disease (nonspecific) Advanced renal failure Normal finding in concentrated urine Heavy proteinuria Urinary tract infection, urinary tract inflammation Urinary tract infection, urinary tract inflammation Acute interstitial nephritis Contaminants Acid urine, acute uric acid nephropathy, hyperuricosuria Alkaline urine Acid urine, hyperoxaluria, ethylene glycol poisoning Cystinuria Sulfa-containing antibiotics

From Andreoli TE (ed): Cecil essentials of medicine, ed 5, Philadelphia, 2001, WB Saunders.

TABLE 4-21

Interpretation of Serologic Tests for Syphilis*

FINDING Nontreponemal Tests

Treponemal Tests

Nonreactive

Nonreactive

Reactive

Nonreactive borderline (FTA-ABS)

Not diagnostic of syphilis: most patients (90%) with this pattern do not develop clinical or serologic evidence of syphilis. Repeat test is indicated. Chronic borderline results are associated with a variety of conditions other than syphilis. Not diagnostic of syphilis. Seen with collagen-vascular disease. Findings diagnostic of syphilis or other treponemal disease. In adequately treated syphilis, one would expect (1) a sustained fourfold drop in titer of reagin test, although reagin test may remain positive after adequate therapy; (2) treponemal tests remain positive after adequate therapy. Concurrent false-positive results on both nontreponemal and treponemal tests could occur in rare instances. It may be impossible to rule out syphilis in an individual with this test profile.

From Stein JH (ed): Internal medicine, ed 4, St Louis, 1994, Mosby. FTA-ABS, Fluorescent treponemal antibody, absorbed. *Serologic data must always be interpreted in the light of a total clinical evaluation. Diagnosis based on serologic criteria alone is fraught with error. Serologic tests apparently in conflict with clinical diagnosis should be confirmed by repetition or possibly referral to a reference laboratory.

LABORATORY TESTS AND INTERPRETATION

Beaded (FTA-ABS) Reactive

Early primary syphilis is not ruled out by negative serologic tests. Early syphilis is present in 13%-30% of patients who have a negative microhemagglutination–Treponema pallidum test; in about 30% of patients who present with chancre but have a nonreactive reagin test; and in about 10% of patients who have a negative FTA-ABS test. Late syphilis is present in a very small fraction of patients. Adequately treated syphilis in remote past may produce these results, but treponemal tests usually remain reactive. Observed in about 10% of patients with chancre. The treponemal tests may turn positive shortly before the reagin tests. Reagin tests repeated after several days are generally positive. In adequately treated early syphilis, the reagin test may return to nonreactive within 1-2 yr, whereas the treponemal tests generally do not. Late syphilis is not ruled out by a negative reagin test. The sensitivity of the reagin tests is lower than that of treponemal tests in untreated late syphilis. In secondary syphilis, rarely, a highly reactive serum appears negative when tested undiluted with a reagin test because flocculation is inhibited by relative antibody excess. Not reported to occur with treponemal tests. Quantitative reagin tests are positive. False-positive treponemal tests occur in 40% of patients with Lyme disease. Finding is not diagnostic of syphilis but constitutes a classic biologic false-positive reaction.

Section IV

Reactive

Interpretation of Finding: Is Syphilis Present?*

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444

Vitamin B12 VITAMIN B12 Normal: 190-900 ng/ml Causes of Vitamin B12 deficiency: 1. Pernicious anemia (antibodies against intrinsic factor and gastric parietal cells) 2. Dietary (strict lacto-ovovegetarians, food faddists) 3. Malabsorption (achlorhydria, gastrectomy, ileal resection, pancreatic insufficiency, drugs [omeprazole, cholestyramine]) Falsely low levels occur in patients with severe folate deficiency, in patients using high doses of ascorbic acid, and when

●

White Blood Count cobalamin levels are measured after nuclear medicine studies (radioactivity interferes with cobalamin radioimmunoassay). Falsely high or normal levels in patients with cobalamin deficiency can occur in severe liver disease and chronic granulocytic leukemia. The absence of anemia or macrocytosis does not exclude the diagnosis of cobalamin deficiency.

WBC; see COMPLETE BLOOD COUNT WHITE BLOOD COUNT; see COMPLETE BLOOD COUNT

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Clinical Preventive Services

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PART A THE PERIODIC HEALTH EXAMINATION* Table 5-1 Ages 65 and older, 447

PART B IMMUNIZATIONS AND CHEMOPROPHYLAXIS Immunizations for adults, 449 Table 5-2A Recommended adult immunization schedule—United States, 449 Table 5-2B Recommended adult immunization schedule for adults with medical conditions—United States, 451 Table 5-3 Immunizing agents and immunization schedules for health-care workers, 452 Table 5-4 Recommendations for persons with medical conditions requiring special vaccination considerations, 455 Administration of vaccines and immune globulins, 456 Table 5-5A Guidelines for spacing live and inactivated antigens, 456 Table 5-5B Guidelines for administering antibodycontaining products and vaccines, 456 Table 5-6 Guide to contraindications and precautions to commonly used vaccines, 457

*Data modified from US Preventive Services Task Force: Guide to clinical preventive services: report of the US Preventive Services Task Force, ed 2,Washington, DC, 1996 (revised 2001), US Department of Health and Human Services. Text downloaded from Internet site: http://text.nlm. nih.gov

Table 5-7 Vaccinations for international travel, 459 Table 5-8 Recommended doses of currently licensed hepatitis B vaccines, 460 Endocarditis prophylaxis, 460 Box 5-1 Cardiac conditions associated with endocarditis, 460 Box 5-2 Dental procedures and endocarditis prophylaxis, 460 Box 5-3 Other procedures and endocarditis prophylaxis, 461 Table 5-9 Prophylactic regimens for dental, oral, respiratory tract, or esophageal procedures, 461 Table 5-10 Prophylactic regimens or genitourinary/ gastrointestinal (excluding esophageal) procedures, 461 Table 5-11 Recommended daily dosage of influenza antiviral medications for treatment and prophylaxis, 462 Postexposure prophylaxis to hepatitis B and HIV, 462 Table 5-12 Recommended postexposure prophylaxis for exposure to hepatitis B virus, 462 Table 5-13 Recommended HIV postexposure prophylaxis for percutaneous injuries, 463 Table 5-14 Recommended HIV postexposure prophylaxis for mucous membrane exposures and nonintact skin exposures, 463 Box 5-4 Situations for which expert consultation for HIV postexposure prophylaxis is advised, 464 Box 5-5 Occupational exposure management resources, 464 Box 5-6 Management of occupational blood exposures, 465 Box 5-7 Basic and expanded HIV postexposure prophylaxis regimens, 466

PART C DISCONTINUATION CONSIDERATIONS Table 5-15 Discontinuation considerations in preventive health screening, 468

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PART A • THE PERIODIC HEALTH EXAMINATION TABLE 5-1

Ages 65 and Older

Interventions considered and recommended for the Periodic Health Examination

Leading causes of death Heart diseases Malignant neoplasms (lung, colorectal, breast) Cerebrovascular disease Chronic obstructive pulmonary disease Pneumonia and influenza INTERVENTIONS

FOR THE

Motorcycle and bicycle helmets* Fall prevention* Safe storage/removal of firearms* Smoke detector* Set hot water heater to 120°-130° F CPR training for household members Dental health Regular visits to dental care provider* Floss, brush with fluoride toothpaste daily* Sexual behavior STD prevention: avoid high-risk sexual behavior*; use condoms

Screening Blood pressure Height and weight Fecal occult blood test1 and/or colonoscopy Mammogram  clinical breast exam2 (women 69 yr) Papanicolaou (Pap) test (women)3 Vision screening Assess for hearing impairment Assess for problem drinking

Counseling Substance use Tobacco cessation Avoid alcohol/drug use while driving, swimming, boating, etc.* Diet and exercise Limit fat and cholesterol; maintain caloric balance; emphasize grains, fruits, vegetables Adequate calcium intake (women) Regular physical activity* Injury prevention Lap/shoulder belts INTERVENTIONS

GENERAL POPULATION

Immunizations Pneumococcal vaccine Influenza1 Tetanus-diphtheria (Td) boosters

FOR

HIGH-RISK POPULATIONS

Population

Potential Interventions (See detailed high-risk definitions)

Institutionalized persons

PPD (HR1); hepatitis A vaccine (HR2); amantadine/rimantadine (HR4) PPD (HR1)

Injection or street drug use

Health care/lab workers Persons susceptible to varicella Men aged 65 to 75 who have ever smoked

Annually. 2Mammogram q1-2 yr, or mammogram q1-2 yr with annual clinical breast exam. 3All women who are or have been sexually active and who have a cervix. Consider discontinuation of testing after age 65 yr if previous regular screening with consistently normal results. *The ability of clinician counseling to influence this behavior is unproven.

Section V

1

Fall prevention intervention (HR5) Consider lipid screening (HR6) Avoid excess/midday sun, use protective clothing* (HR7) PPD (HR1); hepatitis A vaccine (HR2) Hepatitis A vaccine (HR2); hepatitis B vaccine (HR8) HIV screen (HR3); hepatitis B vaccine (HR8) Hepatitis A vaccine (HR2); HIV screen (HR3); hepatitis B vaccine (HR8); RPR/VDRL (HR9) PPD (HR1); hepatitis A vaccine (HR2); HIV screen (HR3); hepatitis B vaccine (HR8); RPR/VDRL (HR9); advice to reduce infection risk (HR10) PPD (HR1); hepatitis A vaccine (HR2); amantadine/rimantadine (HR4); hepatitis B vaccine (HR8) Varicella vaccine (HR11) Ultrasound of abdominal aorta (HR12)

CLINICAL PREVENTIVE SERVICES

Chronic medical conditions; TB contacts; low income; immigrants; alcoholics Persons 75 yr, or 70 yr with risk factors for falls Cardiovascular disease risk factors Family hx of skin cancer; nevi; fair skin, eyes, hair Native Americans/Alaska Natives Travelers to developing countries Blood product recipients High-risk sexual behavior

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The Periodic Health Examination HR1: HIV positive, close contacts of persons with known or suspected TB, health care workers, persons with medical risk factors associated with TB, immigrants from countries with high TB prevalence, medically underserved low-income populations (including homeless), alcoholics, injection drug users, and residents of long-term care facilities. HR2: Persons living in, traveling to, or working in areas where the disease is endemic and where periodic outbreaks occur (e.g., countries with high or intermediate endemicity; certain Alaska Native, Pacific Island, Native American, and religious communities); men who have sex with men; injection or street drug users. Consider for institutionalized persons and workers in these institutions, and day-care, hospital, and laboratory workers. Clinicians should also consider local epidemiology and consider HIV screening in the general population. HR3: Men who had sex with men after 1975; past or present injection drug use; persons who exchange sex for money or drugs, and their sex partners; injection drug–using, bisexual, or HIV-positive sex partner currently or in the past; blood transfusion during 1978-1985; persons seeking treatment for STDs. Clinicians should also consider local epidemiology. HR4: Consider for persons who have not received influenza vaccine or are vaccinated late; when the vaccine may be ineffective due to major antigenic changes in the virus; for unvaccinated persons who provide home care for high-risk persons; to supplement protection provided by vaccine in persons who are expected to have a poor antibody response; and for high-risk persons in whom the vaccine is contraindicated. HR5: Persons aged 75 years and older; or aged 70-74 with one or more additional risk factors including use of certain psychoactive and cardiac medications (e.g., benzodiazepines, anti-

hypertensives); use of 4 prescription medications; impaired cognition, strength, balance, or gait. Intensive individualized home-based multifactorial fall prevention intervention is recommended in settings where adequate resources are available to deliver such services. HR6: Clinicians should consider fasting lipid panel screening on a case-by-case basis for persons aged 65-75, especially in those with additional risk factors (e.g., smoking, diabetes, or hypertension). HR7: Persons with a family or personal history of skin cancer, a large number of moles, atypical moles, poor tanning ability, or light skin, hair, and eye color. HR8: Blood product recipients (including hemodialysis patients), persons with frequent occupational exposure to blood or blood products, men who have sex with men, injection drug users and their sex partners, persons with multiple recent sex partners, persons with other STDs (including HIV), travelers to countries with endemic hepatitis B. HR9: Persons who exchange sex for money or drugs and their sex partners; persons with other STDs (including HIV); and sexual contacts of persons with active syphilis. Clinicians should also consider local epidemiology. HR10: Persons who continue to inject drugs. HR11: Healthy adults without a history of chickenpox or previous immunization. Consider serologic testing for presumed susceptible adults. HR12: Consider ultrasound of abdominal aorta to screen for abdominal aortic aneurysm in all men aged 65 to 75 who have ever smoked.

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449

PART B • IMMUNIZATIONS AND CHEMOPROPHYLAXIS Immunizations for Adults TABLE 5-2, A

Recommended Adult Immunization Schedule—United States Age group (yrs) ⱖ65

Vaccine23 Tetanus, diphtheria (Td)4

1 dose booster every 10 years*

1 dose annually†

Influenza

Pneumococcal (polysaccharide)

1 dose§¶

Hepatitis B4

3 doses (0, 1–2, 4–6 months)**

Hepatitis A

2 doses (0, 6–12 months)††

1 dose if MMR vaccination history is unreliable; 2 doses for persons with occupational or other indications§§

Measles, mumps, rubella (MMR)4

Varicella4

2 doses (0, 4–8 weeks) for persons who are susceptible¶¶

Meningococcal (polysaccharide) For all persons in this age group

1 dose***

For persons with medical/ exposure indications

Catch-up on childhood vaccinations

1

Approved by the Advisory Committee on Immunization Practices and accepted by the American College of Obstetricians and Gynecologists (ACOG) and the American Academy of Family Physicians (AAFP). This schedule indicates recommended age groups for routine administration of currently licensed vaccines for persons aged 19 years. Licensed combination vaccine may be used whenever any components of the combination are indicated and the vaccine’s other components are not contraindicated. Health-care providers should consult manufacturers’ package inserts for detailed recommendations. 3 Additional information regarding these vaccines and contraindications for vaccination is available from the National Immunization Hotline (telephone, 800-232-2522 [English] or 800-232-0233 [Spanish] or at http://www.cdc.gov/nip. 4 Covered by the Vaccine Injury Compensation Program. Information on how to file a claim is available at http://www.hrsa.gov/osp/vicp or by telephone, 800-338-2382. Vaccine injury claims are filed with U.S. Court of Federal Claims, 717 Madison Place, N.W., Washington, D.C. 20005; telephone, 202-219-9657. *Tetanus and diphtheria (Td). Adults should receive a primary series of Td. A primary series for adults is 3 doses: the first 2 doses administered at least 4 weeks apart and the third dose, 6–12 months after the second. Administer 1 dose if the person received the primary series and the last vaccination was 10 years previously. In addition, information is available regarding administration of Td as prophylaxis in wound management (1). The American College of Physicians Task Force on Adult Immunization supports a second option for Td use in adults: a single Td booster at age 50 years for persons who have completed the full pediatric series, including the teenage/young adult booster. †Influenza vaccination. Medical indications: chronic disorders of the cardiovascular or pulmonary systems including asthma; chronic metabolic diseases including diabetes mellitus, renal dysfunction, hemoglobinopathies, or immunosuppression (including immunosuppression caused by medications or by human immunodeficiency virus [HIV]) requiring medical follow-up or hospitalization during the preceding year. Occupational indications: health-care workers (HCWs). Other indications: residents of nursing homes and other long-term-care facilities; persons likely to transmit influenza to persons at high risk (e.g., in-home caregivers to persons with medical indications; household contact and out-of-home caregivers for children aged 23 months, or children with asthma or other indicator conditions for influenza vaccination; household members and caregivers for elderly and adults with high-risk conditions); and anyone who wishes to be vaccinated. §Pneumococcal polysaccharide vaccination. Medical indications: chronic disorders of the pulmonary system, excluding asthma, cardiovascular diseases, diabetes mellitus, chronic liver diseases (including liver disease as a result of alcohol abuse [e.g., cirrhosis]), chronic renal failure or nephrotic syndrome, functional or anatomic asplenia (e.g., sickle cell disease or splenectomy), immunosuppressive conditions (e.g., HIV infection, leukemia, lymphoma, multiple myeloma, Hodgkin’s disease, generalized malignancy, and organ or bone marrow transplantation), chemotherapy with alkylating agents, antimetabolites, or long-term systemic corticosteroids. Geographic/other indications: Alaska Natives and certain American Indian populations. Other indications: residents of nursing homes and other long-term-care facilities (4). ¶Revaccination with pneumococcal polysaccharide vaccine. One-time revaccination after 5 years for persons with chronic renal failure or nephrotic syndrome, functional or anatomic asplenia (e.g., sickle cell disease or splenectomy), immunosuppressive conditions (e.g., HIV infection, leukemia, lymphoma, multiple myeloma, Hodgkin’s disease, generalized malignancy, and organ or bone marrow transplantation), chemotherapy with alkylating agents, antimetabolites, or long-term systemic corticosteroids. For persons aged 65 years, one-time revaccination if they were vaccinated 5 years previously and were aged 65 years at the time of primary vaccination (4). Continued on following page 2

CLINICAL PREVENTIVE SERVICES

Section V

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Immunizations for Adults **Hepatitis B (HepB) vaccine. Medical indications: hemodialysis patients, patients who receive clotting-factor concentrates. Occupational indications: HCWs and public-safety workers who have exposure to blood in the workplace, persons in training in schools of medicine, dentistry, nursing, laboratory technology, and other allied health professions. Behavioral indications: injection-drug users, persons with more than one sex partner during the previous 6 months, persons with a recently acquired sexually transmitted disease (STD), all clients in STD clinics, men who have sex with men (MSM). Other indications: household contacts and sex partners of persons with chronic Hepatitis B virus (HBV) infection, clients and staff of institutions for the developmentally disabled, international travelers to countries with high or intermediate prevalence of chronic HBV infection for 6 months, and inmates of correctional facilities (5). ††Hepatitis A (HepA) vaccine. For the combined HepA-HepB vaccine, use 3 doses (at 0, 1, and 6 months). Medical indications: persons with clotting-factor disorders or chronic liver disease. Behavioral indications: MSM, users of injecting and noninjecting illegal drugs. Occupational indications: persons working with Hepatitis A virus (HAV)-infected primates or with HAV in a research laboratory setting. Other indications: persons traveling to or working in countries that have high or intermediate endemicity of HAV (6). §§Measles, Mumps, Rubella (MMR) vaccination. Measles component: adults born before 1957 might be considered immune to measles. Adults born in or after 1957 should receive at least 1 dose of MMR unless they have a medical contraindication, documentation of at least 1 dose, or other acceptable evidence of immunity. A second dose of MMR is recommended for adults who 1) were exposed recently to measles or were in an outbreak setting, 2) were previously vaccinated with killed measles vaccine, 3) were vaccinated with an unknown vaccine during 1963–1967, 4) are students in postsecondary educational institutions, 5) work in health-care facilities, or 6) plan to travel internationally. Mumps component: 1 dose of MMR should be adequate for protection. Rubella component: Administer 1 dose of MMR to women whose rubella vaccination history is unreliable. ¶¶Varicella vaccination. Recommended for all persons who do not have reliable clinical history of varicella infection, or serologic evidence of varicella zoster virus (VZV) infection who might be at high risk for exposure or transmission. This includes HCWs and family contacts of immunocompromised persons, those who live or work in environments where transmission is likely (e.g., teachers of young children, day-care employees, and residents and staff members in institutional settings), persons who live or work in environments where VZV transmission can occur (e.g., inmates and staff members of correctional institutions, and military personnel) adults living in households with children, and international travelers who are not immune to infection. Approximately 95% of U.S.-born adults are immune to VZV (8,9). ***Meningococcal vaccine (quadrivalent polysaccharide for serogroups A, C, Y, and W-135). Consider vaccination for persons with medical indications: adults with terminal complement component deficiencies or with anatomic or functional asplenia. Other indications: travelers to countries where meningitis is hyperendemic or epidemic (e.g., the “meningitis belt” of sub-Saharan Africa, Mecca, or Saudi Arabia). Revaccination at 3–5 years may be indicated for persons at high risk for infection (e.g., persons residing in areas in which disease is epidemic). Physicians need not initiate discussion of the meningococcal quadrivalent polysaccharide vaccine as part of routine medical care. References 1. CDC. Diphtheria, tetanus, and pertussis: recommendations for vaccine use and other preventive measures. Recommendations of the Immunization Practices Advisory Committee (ACIP). MMWR 1991;40(No. RR-10). 2. CDC. Prevention and control of influenza: recommendations of the Advisory Committee for Immunization Practices. MMWR 2003;52(No. RR-8). 3. CDC. Using live, attenuated influenza vaccine for prevention and control of influenza: supplemental recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 2003;52(No. RR-13). 4. CDC. Prevention of pneumococcal disease: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 1997;47(No. RR-8). 5. CDC. Hepatitis B virus: a comprehensive strategy for eliminating transmission in the United States through universal childhood vaccination. Recommendations of the Immunization Practices Advisory Committee (ACIP). MMWR 1991;40(No. RR-13). 6. CDC. Prevention of hepatitis A through active or passive immunization: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 1999;48(No. RR-12). 7. CDC. Prevention of varicella: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 1996;45(No. RR-11). 8. CDC. Prevention of varicella: updated recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 1999;48(No. RR-6). 9. MMRW Morb Mortal Wkly Rep 52, 2003.

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Recommended Adult Immunization Schedule for Adults with Medical Conditions— United States

TABLE 5-2, B

Vaccine

Medical condition Diabetes, heart disease, chronic pulmonary disease, and chronic liver disease, including chronic alcoholism

Tetanusdiphtheria (Td)*

Influenza†

Pneumococcal (polysaccharide)§¶

A

B

Congenital immunodeficiency, leukemia, lymphoma, generalized malignancy, therapy with alkylating agents, antimetabolites, radiation, or large amounts of corticosteroids

D

Renal failure/end-stage renal disease and patients receiving hemodialysis or clotting factor concentrates

D

Asplenia, including elective splenectomy and terminal complement-component deficiencies Human immunodeficiency virus (HIV) infection For all persons in this group

G

Hepatitis B**

Hepatitis A††

Measles, mumps, rubella (MMR)§§

Varicella¶¶

C

E

F

D,H,I

D,J

For persons with medical/exposure indications

K

Catch-up on childhood vaccinations

Contraindicated

CLINICAL PREVENTIVE SERVICES

From MMWR Morb Mortal Wkly Rep 52, 2003. A. Although chronic liver disease and alcoholism are not indicator conditions for influenza vaccination, administer 1 dose annually if the patient is aged 50 years, has other indications for influenza vaccine, or requests vaccination. B. Asthma is an indicator condition for influenza but not for pneumococcal vaccination. C. For all persons with chronic liver disease. D. For persons aged 65 years, revaccinate once after 5 years have elapsed since initial vaccination. E. Persons with impaired humoral but not cellular immunity may be vaccinated (9). F. For hemodialysis patients use special formulation of vaccine (40 g/ml) or two 1.0 ml 20 g doses administered at one site. Vaccinate early in the course of renal disease. Assess antibody titers to hepatitis B surface antigen (anti-HBs) levels annually. Administer additional doses if anti-HBs levels decline to 10 mlU/ml. G. No data have been reported specifically on risk for severe or complicated influenza infections among persons with asplenia. However, influenza is a risk factor for secondary bacterial infections that might cause severe disease in asplenics. H. Administer meningococcal vaccine and consider Haemophilus influenzae type b vaccine. I. In the event of elective splenectomy, vaccinate 2 weeks before surgery. J. Vaccinate as close to diagnosis as possible when CD4 cell counts are highest. K. Withhold MMR or other measles-containing vaccines from HIV-infected persons with evidence of severe immunosuppression. Please refer to Table 5-2A for footnote explanations.

Section V

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Immunizations for Health-Care Workers TABLE 5-3

Immunizing Agents and Immunization Schedules for Health-Care Workers (HCWs)*

Generic Name

Primary Schedule and Booster(s)

Indications

Immunizing Agents Strongly Recommended for Health-Care Workers Hepatitis B (HB) Two doses IM 4 wk Preexposure: HCWs recombinant apart; third dose 5 at risk for exposure vaccine mo after second; to blood or body booster doses not fluids. necessary.

Hepatitis B immune globulin (HBIG)

0.06 ml/kg IM as soon as possible after exposure. A second dose of HBIG should be administered 1 mo later if the HB vaccine series has not been started.

Influenza vaccine (inactivated whole-virus and split-virus vaccines)

Annual vaccination with current vaccine. Administered IM.

Measles live-virus vaccine

One dose SC; second dose at least 1 mo later.

Mumps live-virus vaccine

One dose SC; no booster

Postexposure prophylaxis: For persons exposed to blood or body fluids containing HBsAg and who are not immune to HBV infection—0.06 ml/ kg IM as soon as possible (but no later than 7 days after exposure). HCWs who have contact with patients at high risk for influenza or its complications; HCWs who work in chronic care facilities; HCWs with high-risk medical conditions or who are aged 65 yr. HCWs† born during or after 1957 who do not have documentation of having received two doses of live vaccine on or after the first birthday or a history of physiciandiagnosed measles or serologic evidence of immunity. Vaccination should be considered for all HCWs who lack proof of immunity, including those born before 1957. HCWs† believed to be susceptible can be vaccinated. Adults born before 1957 can be considered immune.

Major Precautions and Contraindications Previous anaphylactic reaction to common baker’s yeast is a contraindication to vaccination.

Special Considerations The vaccine produces neither therapeutic nor adverse effects on HBV-infected persons. Prevaccination serologic screening is not indicated for persons being vaccinated because of occupational risk. HCWs who have contact with patients or blood should be tested 1-2 mo after vaccination to determine serologic response.

History of anaphylactic hypersensitivity to egg ingestion.

Immunocompromised persons,‡ including HIV-infected persons who have evidence of severe immunosuppression; anaphylaxis after gelatin ingestion or administration of neomycin; recent administration of immune globulin.

MMR is the vaccine of choice if recipients are likely to be susceptible to rubella and/or mumps as well as to measles. Persons vaccinated during 1963-1967 with a killed measles vaccine alone, killed vaccine followed by live vaccine, or with a vaccine of unknown type should be revaccinated with two doses of live measles virus vaccine.

Immunocompromised persons‡; history of anaphylactic reaction after gelatin ingestion or administration of neomycin

MMR is the vaccine of choice if recipients are likely to be susceptible to measles and rubella as well as to mumps.

Modified from MMWR Morb Mortal Wkly Rep 46(RR-18), 1998. HBsAg, Hepatitis B surface antigen; HBV, hepatitis B virus; HIV, human immunodeficiency virus; IM, intramuscular; MMR, measles, mumps, rubella vaccine; SC, subcutaneous. *Persons who provide health care to patients or work in institutions that provide patient care (e.g., physicians, nurses, emergency medical personnel, dental professionals and students, medical and nursing students, laboratory technicians, hospital volunteers, and administrative and support staff in health-care institutions). †All HCWs (i.e., medical or nonmedical, paid or volunteer, full time or part time, student or nonstudent, with or without patient-care responsibilities) who work in healthcare institutions (e.g., inpatient and outpatient, public and private) should be immune to measles, rubella, and varicella. ‡Persons immunocompromised because of immune deficiency diseases, HIV infection, leukemia, lymphoma, or generalized malignancy or immunosuppressed as a result of therapy with corticosteroids, alkylating drugs, antimetabolites, or radiation.

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Immunizing Agents and Immunization Schedules for Health-Care Workers (HCWs)* (Continued)

TABLE 5-3

Generic Name

Primary Schedule and Booster(s)

Indications

Hepatitis A vaccine

Two doses of vaccine either 6-12 mo apart (HAVRIX), or 6 mo apart (VAQTA)

Not routinely indicated for HCWs in the United States. Persons who work with HAVinfected primates or with HAV in a research laboratory setting should be vaccinated.

Meningococcal polysaccharide vaccine (tetravalent A, C, W135, and Y) Typhoid vaccine, IM, SC, and oral

One dose in volume and by route specified by manufacturer; need for boosters unknown IM vaccine: One 0.5-ml dose, booster 0.5 ml every 2 yr. SC vaccine: two 0.5 ml doses, 4 wk apart, booster 0.5 ml SC or 0.1 ID every 3 yr if exposure continues Oral vaccine: Four doses on alternate days. The manufacturer recommends revaccination with the entire four-dose series every 5 yr One dose administered with a bifurcated needle; boosters administered every 10 yr

Not routinely indicated for HCWs in the United States.

Vaccinia vaccine (smallpox)

Special Considerations

History of anaphylactic hypersensitivity to alum or, for HAVRIX, the preservative 2-phenoxyethanol. The risk associated with vaccination should be weighed against the risk for hepatitis A in women who may be at high risk for exposure to HAV.

Workers in microbiology laboratories who frequently work with Salmonella typhi

Severe local or systemic reaction to a previous dose. Ty21a (oral) vaccine should not be administered to immunocompromised persons† or to persons receiving antimicrobial agents.

Vaccination should not be considered an alternative to the use of proper procedures when handling specimens and cultures in the laboratory.

Laboratory workers who directly handle cultures with vaccinia, recombinant vaccinia viruses, or orthopox viruses that infect humans

The vaccine is contraindicated in persons with eczema or a history of eczema and in immunocompromised persons† and their household contacts.

Vaccination may be considered for HCWs who have direct contact with contaminated dressings or other infectious material from volunteers in clinical studies involving recombinant vaccinia virus.

All adults

History of a neurologic reaction or immediate hypersensitivity reaction after a previous dose. History of severe local (Arthus-type) reaction after a previous dose. Such persons should not receive further routine or emergency doses of Td for 10 yr.

Tetanus prophylaxis in wound management‡

*Persons who provide health care to patients or work in institutions that provide patient care (e.g., physicians, nurses, emergency medical personnel, dental professionals and students, medical and nursing students, laboratory technicians, hospital volunteers, and administrative and support staff in health-care institutions). †All HCWs (i.e., medical or nonmedical, paid or volunteer, full time or part time, student or nonstudent, with or without patient-care responsibilities) who work in healthcare institutions (e.g., inpatient and outpatient, public and private) should be immune to measles, rubella, and varicella. ‡Persons immunocompromised because of immune deficiency diseases, HIV infection, leukemia, lymphoma or generalized malignancy or immunosuppressed as a result of therapy with corticosteroids, alkylating drugs, antimetabolites, or radiation. Continued on following page

CLINICAL PREVENTIVE SERVICES

Other Vaccine-Preventable Diseases Tetanus and Two IM doses 4 wk diphtheria apart; third dose 6-12 (toxoids [Td]) mo after second dose; booster every 10 yr

Major Precautions and Contraindications

Section V

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Immunizations for Health-Care Workers Immunizing Agents and Immunization Schedules for Health-Care Workers (HCWs)* (Continued)

TABLE 5-3

Generic Name

Primary Schedule and Booster(s)

Pneumococcal polysaccharide vaccine (23 valent)

One dose, 0.5 ml, IM or SC; revaccination recommended for those at highest risk 5 yr after the first dose

Rubella live-virus vaccine

One dose SC; no booster

Varicella zoster livevirus vaccine

Two 0.5-ml doses SC 4-8 wk apart if 13 yr of age

Varicella-zoster immune globulin (VZIG)

Persons 50 kg: 125 /10 kg IM; persons 50 kg: 625 §

BCG Vaccination Bacille CalmetteGuérin (BCG) vaccine (tuberculosis)

Indications Adults who are at increased risk of pneumococcal disease and its complications because of underlying health conditions; older adults, especially those age 65 who are healthy Indicated for HCWs,† both men and women, who do not have documentation of having received live vaccine on or after their first birthday or laboratory evidence of immunity. Adults born before 1957 can be considered immune. Indicated for HCWs† who do not have either a reliable history of varicella or serologic evidence of immunity

Major Precautions and Contraindications Previous recipients of any type of pneumococcal polysaccharide vaccine who are at highest risk for fatal infection or antibody loss may be revaccinated 5 yr after the first dose. Immunocompromised persons†; history of anaphylactic reaction after administration of neomycin

MMR is the vaccine of choice if recipients are likely to be susceptible to measles or mumps, as well as to rubella.

Immunocompromised persons,‡ history of anaphylactic reaction following receipt of neomycin or gelatin. Avoid salicylate use for 6 wk after vaccination.

Vaccine is available from the manufacturer for certain patients with acute lymphocytic leukemia (ALL) in remission. Because 71%-93% of persons without a history of varicella are immune, serologic testing before vaccination is likely to be costeffective. Serologic testing may help in assessing whether to administer VZIG. If use of VZIG prevents varicella disease, patient should be vaccinated subsequently.

Persons known or likely to be susceptible (particularly those at high risk for complications) who have close and prolonged exposure to a contact case or to an infectious hospital staff worker or patient

One percutaneous dose of 0.3 ml; no booster dose recommended

Should be considered Should not be adminisonly for HCWs in tered to immunocomareas where multipromised persons‡ drug tuberculosis is prevalent, a strong likelihood of infection exists, and where comprehensive infection control precautions have failed to prevent TB transmission to HCWs Other Immunobiologics That Are or May Be Indicated for Health-Care Workers Immune globulin Postexposure—One Indicated for HCWs Contraindicated in (hepatitis A) IM dose of 0.02 exposed to feces of persons with IgA ml/kg administered infectious patients deficiency; do not 2 wk after administer within 2 exposure wk after MMR vaccine, or 3 wk after varicella vaccine. Delay administration of MMR vaccine for 3 mo and varicella vaccine 5 mo after administration of IG

Modified from MMWR Morb Mortal Wkly Rep 46(RR-18), 1998. §Some experts recommend 125 /10 kg regardless of total body weight.

Special Considerations

In the United States tuberculosis-control efforts are directed toward early identification, treatment of cases, and preventive therapy with isoniazid.

Administer in large muscle mass (deltoid, gluteal).

Contrf Contrf

Rou/Contre Contr Rou Rou Rou Rou Rou Rou Rou Rou Rou

Rou Rou

Rou Rou Rou Rou Rou Rou Rou

Rou

Rou

HAV Rou Rou Rou Rou Rec Rou Rou Rou Rou Rou Rec

HBV Roug Roug Recg Rou Rou Rou Rou Rou Rou Rou Rec

Rou

Rou

Rec Rec Rec Rec Rec Rec Recj

Rec Rec

Pneumovaxa

Rou

Rou

Rec Rec Rec Rec Rec Rec Rec

Rec Rec

Influenzab

Rou

Rou

Rou Rou Rou Rou Rou Rou Recj

Cons Roui

HbCV

Rou

Rec

Rou Rou Rou Rou Rou Rou Recj

Rou Rou

Meningococcal

Rou

Rou

Rou Rou Rou Rou Rou Rou Rou

Rou Rou

IPV

Rou

Rou

Rou Rou Rou Rou Rou Rou Rou

Contr Contr

Other live vaccinesc

Rou

Rou Rou Rou Rou Rou Rou Rou

Rou Rou

Other Killed Vaccinesd

Immunizations for Adults

CLINICAL PREVENTIVE SERVICES

Section V

Modified and updated from MMWR Morb Mortal Wkly Rep 42(RR-4):16 and 17, 1993. Cons, Consider vaccination; Contr, contraindicated; Rec, recommended; Rou, routine as outlined for all adults. a Pneumovax should be repeated in 5 years for patients in whom vaccine is recommended. Asthma without chronic obstructive pulmonary disease is not an indication for the vaccine. b Influenza vaccine should also be given to caregivers and household members. c Includes bacille Calmette-Guérin, vaccinia, oral typhoid, yellow fever (if exposure cannot be avoided, persons with HIV can be given yellow fever vaccine; see text). d Includes rabies (check postvaccination titers in HIV or severely immunocompromised persons), Lyme, inactivated typhoid, cholera, plague, and anthrax. e For asymptomatic, nonseverely immunocompromised persons with human immunodeficiency virus (HIV), MMR can be used; it is contraindicated in severely immunocompromised persons. MMR can be considered in symptomatic HIV patients without severe immunocompromise. f Varicella can be given to household members and caregivers, but if varicella-like rash develops after vaccination, contact should be avoided. g Recommended for persons with severe chronic renal failure approaching or already receiving dialysis, and higher doses should be given. Antibody titers should be measured after vaccination in these patients and in those with HIV or severe immunocompromise (who may require higher doses) to ensure adequate response. Yearly titers should be measured in dialysis patients. h Severe immunocompromise can result from congenital immunodeficiency, leukemia, lymphoma, malignancy, organ transplant, chemotherapy, radiation therapy, or high-dose corticosteroids. i Only for persons with Hodgkin’s disease. j Give at least 2 weeks in advance of elective splenectomy.

Rou

Rou

Rou Rou Rou Rou Rou Rou Rou

Varicella

MMR

Td

Recommendations for Persons with Medical Conditions Requiring Special Vaccination Considerations

HIV infection Severe immunocompromiseh Renal failure Diabetes Chronic liver disease Cardiac disease Pulmonary disease Alcoholism Functional/anatomic asplenia Terminal complement deficiency Clotting factor disorders

Condition

TABLE 5-4

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Administration of Vaccines and Immune Globulins TABLE 5-5, A

Guidelines for Spacing of Live and Inactivated Antigens

Antigen Combination

Recommended Minimum Interval Between Doses

2 inactivated Inactivated and live 2 live parenteral*

None; can be administered simultaneously or at any interval between doses None; can be administered simultaneously or at any interval between doses 4-week minimum interval, if not administered simultaneously

From MMWR Morb Mortal Wkly Rep 51(RR-2), 2002. *Live oral vaccines (e.g., Ty21a typhoid vaccine, oral polio vaccine) can be administered simultaneously or at any interval before or after inactivated or live parenteral vaccines.

TABLE 5-5, B

Guidelines for Administering Antibody-Containing Products* and Vaccines

Simultaneous Administration Combination

Recommended Minimum Interval Between Doses

Antibody-containing products and inactivated antigen

None; can be administered simultaneously at different sites or at any time between doses Should not be administered simultaneously,† if simultaneous administration of measles-containing vaccine or varicella vaccine is unavoidable, administer at different sites and revaccinate or test for seroconversion after the recommended interval

Antibody-containing products and live antigen

Nonsimultaneous Administration Product Administered First

Second

Recommended Minimum Interval Between Doses

Antibody-containing products Inactivated antigen Antibody-containing products Live antigen

Inactivated antigen Antibody-containing products Live antigen Antibody-containing products

None None Dose-related‡ 2 weeks

From MMWR Morb Mortal Wkly Rep 51(RR-2), 2002. *Blood products containing substantial amounts of immunoglobulin, including intramuscular and intravenous immune globulin, specific hyperimmune globulin (e.g., hepatitis B immune globulin, tetanus immune globulin, varicella zoster immune globulin, and rabies immune globulin), whole blood, packed red cells, plasma, and platelet products. †Yellow fever and oral Ty21a typhoid vaccines are exceptions to these recommendations. These live attenuated vaccines can be administered at any time before, after, or simultaneously with an antibody-containing product without substantially decreasing the antibody response. ‡The duration of interference of antibody-containing products with the immune response to the measles component of measles-containing vaccine, and possibly varicella vaccine, is dose-related.

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Administration of Vaccines and Immune Globulins TABLE 5-6

Guide to Contraindications and Precautionsa to Commonly Used Vaccines

Vaccine

True Contraindications and Precautionsa

Untrue (Vaccines Can Be Administered)

General for all vaccines, including diphtheria and tetanus toxoids; adult tetanus-diphtheria toxoid (Td); inactivated poliovirus vaccine (IPV); measles-mumps-rubella vaccine (MMR); hepatitis A vaccine; hepatitis B vaccine; varicella vaccine; pneumococcal conjugate vaccine (PCV); influenza vaccine; and pneumococcal polysaccharide vaccine (PPV)

Contraindications Serious allergic reaction (e.g., anaphylaxis) after a previous vaccine dose Serious allergic reaction (e.g., anaphylaxis) to a vaccine component Precautions Moderate or severe acute illness with or without fever

DTaP

Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precautions Moderate or severe acute illness with or without fever

Mild acute illness with or without fever Mild to moderate local reaction (i.e., swelling, redness, soreness); low-grade or moderate fever after previous dose Lack of previous physical examination in well-appearing person Current antimicrobial therapy Convalescent phase of illness Recent exposure to an infectious disease History of penicillin allergy, other nonvaccine allergies, relatives with allergies, receiving allergen extract immunotherapy Temperature of 40.5° C, fussiness or mild drowsiness after a previous dose of diphtheria toxoid-tetanus toxoid-pertussis vaccine (DTP)/DTaP Family history of seizures Stable neurologic conditions (e.g., cerebral palsy, well-controlled convulsions, developmental delay)

DT, Td

Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precautions Guillain-Barré syndrome 6 wk after previous dose of tetanus toxoid-containing vaccine Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction to previous dose or vaccine component Precautions Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Known severe immunodeficiency (e.g., hematologic and solid tumors; congenital immunodeficiency; long-term immunosuppressive therapy,c or severely symptomatic human immunodeficiency virus [HIV] infection) Precautions Recent (11 mo) receipt of antibody-containing blood product (specific interval depends on product) History of thrombocytopenia or thrombocytopenic purpura Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Age 6 wk Precaution Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precautions Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precautions Moderate or severe acute illness with or without fever

IPV

MMRb

Hepatitis B

Autoimmune disease (e.g., systemic lupus erythematosus or rheumatoid arthritis)

Section V

Hepatitis A

Positive tuberculin skin test Simultaneous TB skin testingd Immunodeficient family member or household contact Asymptomatic or mildly symptomatic HIV infection Allergy to eggs

CLINICAL PREVENTIVE SERVICES

Hib

457

Continued on following page

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Administration of Vaccines and Immune Globulins TABLE 5-6

Guide to Contraindications and Precautionsa to Commonly Used Vaccines (Continued)

Vaccine

True Contraindications and Precautionsa

Untrue (Vaccines Can Be Administered)

Varicellab

Contraindications Severe allergic reaction after a previous dose or to a vaccine component Substantial suppression of cellular immunity Precautions Recent (11 mo) receipt of antibody-containing blood product (specific interval depends on product) Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precaution Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction to previous dose or vaccine component, including egg protein Precautions Moderate or severe acute illness with or without fever Contraindications Severe allergic reaction after a previous dose or to a vaccine component Precaution Moderate or severe acute illness with or without fever

Immunodeficient family member or household contacte Asymptomatic or mildly symptomatic HIV infection Humoral immunodeficiency (e.g., agammaglobulinemia)

PCV

Influenza

PPV

Nonsevere (e.g., contact) allergy to latex or thimerosal Concurrent administration of Coumadin or aminophylline

From MMWR Morb Mortal Wkly Rep 51(RR-2), 2002. a Events or conditions listed as precautions should be reviewed carefully. Benefits and risks of administering a specific vaccine to a person under these circumstances should be considered. If the risk from the vaccine is believed to outweigh the benefit, the vaccine should not be administered. If the benefit of vaccination is believed to outweigh the risk, the vaccine should be administered. b MMR and varicella vaccines can be administered on the same day. If not administered on the same day, these vaccines should be separated by 28 days. c Substantially immunosuppressive steroid dose is considered to be 2 weeks of daily receipt of 20 mg or 2 mg/kg body weight of prednisone or equivalent. d Measles vaccination can suppress tuberculin reactivity temporarily. Measles-containing vaccine can be administered on the same day as tuberculin skin testing. If testing cannot be performed until after the day of MMR vaccination, the test should be postponed for 4 weeks after the vaccination. If an urgent need exists to skin test, do so with the understanding that reactivity might be reduced by the vaccine. e If a vaccinee experiences a presumed vaccine-related rash 7-25 days after vaccination, avoid direct contact with immunocompromised persons for the duration of the rash.

Africa, Asia, and Americas in rural mountainous or upland areas.

Parenteral vaccine series, 28 day (see text) Anamnestic response to booster Oral vaccine, 7 days Parenteral vaccine, probably 14 days 10 days

All travelers; vaccine series/booster.

Probably 30 days

If required by local authorities, one dose usually suffices; primary series only for those living in high-risk areas under poor sanitary conditions or those with compromised gastric defense mechanisms (achlorhydria, antacid therapy, previous ulcer surgery); booster every 6 mo. Travelers whose research or field activities bring them in contact with rodents; vaccine series/booster; consider taking tetracycline (500 mg four times a day) for chemoprophylaxis (inferred from clinical experience in treating plague).

Probably 30 days

10 days

Travelers staying for more than 30 days in high-risk rural areas; staying outdoors during transmission season; vaccine series.

All travelers; vaccine/booster at approved yellow fever vaccination center.

Travelers with prolonged stay in rural areas with poor sanitation; vaccine series/booster.

7-14 days

Travelers staying for more than 30 days or at high risk of exposure to domestic or wild animals; vaccine series/booster.

7-10 days

Vaccine, 30 days Pooled IG, 2 days 7-14 days

Probably 30 days for series Anamnestic response to booster 7-14 days Probably 30 days

Ideal Time Between Last Vaccine Dose and Travel

Administration of Vaccines for Travel

CLINICAL PREVENTIVE SERVICES

Section V

From Noble J: Primary care medicine, ed 3, St Louis, 2001, Mosby. *Only yellow fever vaccine is required for entry by any country; cholera vaccine may be required by some local authorities; and meningococcus vaccine is required for pilgrims to Mecca, Saudia Arabia, during Haj. However, it is important to follow CDC recommendations for all vaccines to prevent disease. If a required vaccine is contraindicated or withheld for any reason, attempts should be made to obtain a waiver from the country’s consulate or embassy. †Because areas affected can change, and for more specific details, consult CDC’s traveler’s hotline.

Plague

Cholera*

Japanese encephalitis

Yellow fever*

Typhoid fever

Poliomyelitis

Rabies

Meningococcus*

North and central South America, forest-savannah zones of Africa; some countries in Africa, Asia, and Middle East require travelers from endemic areas to be vaccinated. Seasonally in most areas of Asia, Indian subcontinent, and western Pacific islands; in temperate zones, incidence increases in summer and early fall; in tropics, year-round incidence. Certain undeveloped countries.

Tropics throughout the year; Southern Hemisphere from April to September. Sub-Saharan Africa “belt” (Senegal to Ethiopia) from December to June; required for pilgrims to Saudi Arabia during Haj; epidemics reported in other African nations, India, Nepal, and Mongolia. Endemic dog rabies exists in Mexico, El Salvador, Guatemala, Peru, Colombia, Ecuador, India, Nepal, Philippines, Sri Lanka, Thailand, and Vietnam. Developing countries not in Western Hemisphere; at risk all year in tropics; in temperate zones, incidence increases in summer and fall. Many countries in Asia, Africa, Central America, and South America.

Influenza

Travelers to rural areas; eating and drinking in settings of poor sanitation; vaccine or pooled immune globulin (IG). Travelers for whom vaccine is otherwise indicated; give current vaccine and revaccinate in fall as usual. All travelers; vaccine.

All travelers; antibody titer, reported illness, or vaccine series. Travelers for more than 6 mo in close contact with population or for less time but with high-risk activities (close household contact, seeking dental or medical care, sex); vaccine series.

All 5%-20% of population are carriers in Africa, Middle East except Israel, all Southeast Asia, Amazon basin, Haiti, and Dominican Republic; 1%-5% of population are carriers in south-central and southwest Asia, Israel, Japan, Americas, Russia, and eastern and southern Europe. Developing countries.

Varicella Hepatitis B

Hepatitis A

All travelers; vaccine series/booster.

All

Tetanus

Prophylaxis Recommended

Areas Affected†

Vaccinations for International Travel

Disease*

TABLE 5-7

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Hepatitis/Endocarditis Prophylaxis TABLE 5-8

Recommended Doses of Currently Licensed Hepatitis B Vaccines

Population Group

Recombivax HB* Dose in g (Dose in ml)

Engerix-B* Dose in mg (Dose in ml)

Adults 20 yr Dialysis patients and other immunocompromised persons

10 (1.0) 40†

20 (1.0) 40‡

Modified from MMWR Morb Mortal Wkly Rep 40(RR-13):7, 1991. *Both vaccines are routinely administered in a three-dose series at 0, 1, and 6 mo. Engerix-B is also licensed for a four-dose series administered at 0, 1, 2, and 12 mo. †Special formulation. ‡Two 1.0-ml doses administered at one site in a four-dose schedule at 0, 1, 2, and 6 mo.

ENDOCARDITIS PROPHYLAXIS BOX 5-1

Cardiac Conditions Associated with Endocarditis

Endocarditis Prophylaxis Recommended High-Risk Category Prosthetic cardiac valves, including bioprosthetic and homograft valves Previous bacterial endocarditis Complex cyanotic congenital heart disease (e.g., single ventricle states, transposition of the great arteries, tetralogy of Fallot) Surgically constructed systemic pulmonary shunts or conduits Moderate-Risk Category Most other congenital cardiac malformations (other than above and below) Acquired valvar dysfunction (e.g., rheumatic heart disease) Hypertrophic cardiomyopathy Mitral valve prolapse with valvar regurgitation and/or thickened leaflets

Endocarditis Prophylaxis Not Recommended Negligible-Risk Category (No Greater Risk Than the General Population) Isolated secundum atrial septal defect Surgical repair of atrial septal defect, ventricular defect, or patent ductus arteriosus (without residua beyond 6 mos) Previous coronary artery bypass graft surgery Mitral valve prolapse without valvar regurgitation Physiologic, functional, or innocent heart murmurs Previous Kawasaki disease without valvar dysfunction Previous rheumatic fever without valvar dysfunction Cardiac pacemakers (intravascular and epicardial) and implanted defibrillators

From Dajani AS et al: JAMA 277:1794-1801, 1997.

BOX 5-2

Dental Procedures and Endocarditis Prophylaxis

Endocarditis Prophylaxis Recommended* Dental extractions Periodontal procedures including surgery, scaling and root planing, probing, and recall maintenance Dental implant placement and reimplantation of avulsed teeth Endodontic (root canal) instrumentation of surgery only beyond the apex Subgingival placement of antibiotic fibers or strips Initial placement of orthodontic bands—but not brackets Intraligamentary local anesthetic injections Prophylactic cleaning of teeth or implants where bleeding is anticipated

Endocarditis Prophylaxis Not Recommended Restorative dentistry† (operative and prosthodontic) with or without retraction cord‡ Local anesthetic injections (nonintraligamentary) Intracanal endodontic treatment; postplacement and buildup Placement of rubber dams Postoperative suture removal Placement of removable prosthodontic or orthodontic appliances Taking of oral impressions Fluoride treatments Taking of oral radiographs Orthodontic appliance adjustment Shedding of primary teeth

From Dajani AS et al: JAMA 277:1794-1801, 1997. *Prophylaxis is recommended for patients with high- and moderate-risk cardiac conditions. †This includes restoration of decayed teeth (filling cavities) and replacement of missing teeth. ‡Clinical judgment may indicate antibiotic use in selected circumstances that may create significant bleeding.

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Endocarditis Prophylaxis BOX 5-3

461

Other Procedures and Endocarditis Prophylaxis

Endocarditis Prophylaxis Recommended Respiratory tract Tonsillectomy and/or adenoidectomy Surgical operations that involve respiratory mucosa Bronchoscopy with a rigid bronchoscope Gastrointestinal Tract* Sclerotherapy for esophageal varices Esophageal stricture dilation Endoscopic retrograde cholangiography* with biliary obstruction Biliary tract surgery Surgical operations that involve intestinal mucosa Genitourinary Tract Prostatic surgery Cystoscopy Urethral dilation Endocarditis Prophylaxis Not Recommended Respiratory Tract Endotracheal intubation Bronchoscopy with a flexible bronchoscope, with or without biopsy† Tympanostomy tube insertion

Gastrointestinal Tract Transesophageal echocardiography† Endoscopy with or without gastrointestinal biopsy† Genitourinary Tract Vaginal hysterectomy† Vaginal delivery† Cesarean section In uninfected tissue: Urethral catheterization Uterine dilation and curettage Therapeutic abortion Sterilization procedures Insertion or removal of intrauterine devices Other Cardiac catheterization, including balloon angioplasty Implanted cardiac pacemakers, implanted defibrillators, and coronary stents Incision or biopsy of surgically scrubbed skin Circumcision

From Dajani AS et al: JAMA 277:1794-1801, 1997. *Prophylaxis is recommended for high-risk patients; optional for medium-risk patients. †Prophylaxis is optional for high-risk patients.

TABLE 5-9

Prophylactic Regimens for Dental, Oral, Respiratory Tract, or Esophageal Procedures

Situation

Agent

Regimen

Standard general prophylaxis Unable to take oral medications Allergic to penicillin

Amoxicillin Ampicillin Clindamycin or Cephalexin* or cefadroxil* or Azithromycin or clarithromycin Clindamycin or Cefazolin*

2.0 g 2.0 g intramuscularly (IM) or intravenously (IV) 600 mg

Allergic to penicillin and unable to take oral medications

2.0 g 500 mg 600 mg 1.0 g

From Dajani AS et al: JAMA 277:1794-1801, 1997. *Cephalosporins should not be used in individuals with immediate-type hypersensitivity reaction (urticaria, angioedema, or anaphylaxis) to penicillins.

Prophylactic Regimens for Genitourinary/Gastrointestinal (Excluding Esophageal) Procedures

Situation

Agents

Regimen*

High-risk patients

Ampicillin plus gentamicin

High-risk patients allergic to ampicillin Moderate-risk patients

Vancomycin plus gentamicin Amoxicillin or ampicillin Vancomycin

Ampicillin 2.0 g intramuscularly (IM) or intravenously (IV) plus gentamicin 1.5 mg/kg (not to exceed 120 mg) within 30 min of starting the procedure; 6 hr later, ampicillin 1 g IM/IV or amoxicillin 1 g orally (PO) Vancomycin 1.0 g IV over 1-2 hr plus gentamicin 1.5 mg/kg IV/IM (not to exceed 120 mg); complete injection/infusion within 30 min of starting the procedure Amoxicillin 2.0 g PO 1 hr before procedure, or ampicillin 2.0 g IV/IV within 30 min of starting the procedure Vancomycin 1.0 g IV over 1-2 hr; complete infusion within 30 min of starting the procedure

Moderate-risk patients allergic to ampicillin/amoxicillin

Section V

From Dajani AS et al: JAMA 277:1794-1801, 1997. *No second dose of vancomycin or gentamicin is recommended.

CLINICAL PREVENTIVE SERVICES

TABLE 5-10

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Influenza Treatment and Prophylaxis/Occupational Exposure TABLE 5-11 Recommended Daily Dosage of Influenza Antiviral Medications for Treatment and Prophylaxis Antiviral Agent

65 Years and Older

a

Amantadine Treatment Prophylaxis Rimantadineb Treatmentc Prophylaxis Zanamivire,f Treatment Oseltamivir Treatmentg Prophylaxis

100 mg or less per day 100 mg or less per day 100 or 200d mg per day 100 or 200d mg per day 10 mg bid 75 mg bid 75 mg per day

Modified from MMWR Morb Mortal Wkly Rep 50(RR-4):1, 2001. NA, Not applicable. a The drug package insert should be consulted for dosage recommendations for administering amantadine to persons with creatinine clearance of 50 ml or less per min per 1.73m2. b A reduction in dosage to 100 mg per day of rimantadine is recommended for persons who have severe hepatic dysfunction or those with creatinine clearance of 10 ml or less per min. Other persons with less severe hepatic or renal dysfunction taking 100 mg per day of rimantadine should be observed closely, and the dosage should be reduced or the drug discontinued, if necessary. c Only approved for treatment in adults. d Elderly residents of nursing homes should be administered only 100 mg per day of rimantadine. A reduction in dosage of 100 mg per day should be considered for all persons 65 yr of age or older if they experience side effects when taking 200 mg per day. e Zanamivir is administered via inhalation by using a plastic device included in the package with the medication. Patients will benefit from instruction and demonstration of correct use of the device. f Zanamivir is not approved for prophylaxis. g A reduction in the dose of oseltamivir is recommended for persons with creatinine clearance of less than 30 ml per min.

TABLE 5-12

Recommended Postexposure Prophylaxis for Exposure to Hepatitis B Virus

Vaccination and Antibody Response Status of Exposed Workers* Unvaccinated Previously vaccinated Known responder§ Known nonresponderi Antibody response unknown

TREATMENT Source HBsAg Positive

Source HBsAg Negative

Source Unknown or Not Available for Testing

HBIG†  1 and initiate HB vaccine series‡

Initiate HB vaccine series

Initiate HB vaccine series

No treatment HBIG  1 and initiate revaccination or HBIG  2¶ Test exposed person for anti-HBs¶ 1. If adequate,§ no treatment is necessary 2. If inadequate, administer HBIG  1 and vaccine booster

No treatment No treatment

No treatment If known high-risk source, treat as if source were HBsAg positive Test exposed person for anti-HBs 1. If adequate,‡ no treatment is necessary 2. If inadequate,‡ administer vaccine booster and recheck titer in 1-2 mo

No treatment

Anti-HBs, Antibody to HBsAg; HB, hepatitis B; HBIG, hepatitis B immune globulin; HBsAg, hepatitis B surface antigen. *Persons who have previously been infected with HBV are immune to reinfection and do not require postexposure prophylaxis. †Hepatitis B immune globulin; dose is 0.06 ml/kg intramuscularly. ‡Hepatitis B vaccine. §A responder is a person with adequate levels of serum antibody to HBsAg (i.e., anti-HBs 10 mlU/ml). A nonresponder is a person with inadequate response to vaccination (i.e., serum anti-HBs 10 mlU/ml). ¶The option of giving one dose of HBIG and reinitiating the vaccine series is preferred for nonresponders who have not completed a second 3-dose vaccine series. For persons who previously completed a second vaccine series but failed to respond, two doses of HBIG are preferred.

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Occupational Exposure/HIV Postexposure Prophylaxis TABLE 5-13

463

Recommended HIV Postexposure Prophylaxis for Percutaneous Injuries INFECTION STATUS OF SOURCE

HIV-Positive Exposure Type Class 1*

HIV-Positive Class 2*

Source of Unknown HIV Status†

Less severe§

Recommend basic 2-drug PEP

Recommend expanded 3-drug PEP

Generally, no PEP warranted; however, consider basic 2drug PEP for source with HIV risk factors††

More severe#

Recommend expanded 3-drug PEP

Recommend expanded 3-drug PEP

Generally, no PEP warranted; however, consider basic 2drug PEP for source with HIV risk factors¶

Unknown Source‡

HIV-Negative

Generally, no PEP warranted; however, consider basic 2drug PEP in settings where exposure to HIV-infected persons is likely Generally, no PEP warranted; however, consider basic 2-drug PEP in settings where exposure to HIVinfected persons is likely

No PEP warranted

No PEP warranted

HIV, Human immunodeficiency virus; PEP, postexposure prophylaxis (see Box 5-7). *HIV-Positive, Class 1—asymptomatic HIV infection or known low viral load (e.g., 1500 RNA copies/ml). HIV-Positive, Class 2—symptomatic HIV infection, acquired immunodeficiency syndrome, acute seroconversion, or known high viral load. If drug resistance is a concern, obtain expert consultation. Initiation of PEP should not be delayed pending expert consultation, and, because expert consultation alone cannot substitute for face-to-face counseling, resources should be available to provide immediate evaluation and follow-up care for all exposures. †Source of unknown HIV status (e.g., deceased source person with no samples available for HIV testing). ‡Unknown source (e.g., a needle from a sharps disposal container). §Less severe (e.g., solid needle and superficial injury). The designation “consider PEP” indicates that PEP is optional and should be based on an individualized decision between the exposed person and the treating clinician. ¶If PEP is offered and taken and the source is later determined to be HIV-negative, PEP should be discontinued. #More severe (e.g., large-bore hollow needle, deep puncture, visible blood on device, or needle used in patient’s artery or vein).

TABLE 5-14 Recommended HIV Postexposure Prophylaxis for Mucous Membrane Exposures and Nonintact Skina Exposures INFECTION STATUS OF SOURCE

Exposure Type Small volume

e

Large volumeh

HIV-Positive Class 1b

HIV-Positive Class 2b

Consider basic 2-drug PEPf

Recommend basic 2-drug PEP

Recommend basic 2-drug PEP

Recommend expanded 3-drug PEP

Source of Unknown HIV Statusc

Unknown Sourced

HIV-Negative

Generally, no PEP warranted; however, consider basic 2drug PEPf for source with HIV risk factorsg Generally, no PEP warranted; however, consider basic 2drug PEPf for source with HIV risk factorsg

Generally, no PEP warranted; however, consider basic 2-drug PEPf in settings where exposure to HIVinfected persons is likely Generally, no PEP warranted; however, consider basic 2-drug PEPf in settings where exposure to HIVinfected persons is likely

No PEP warranted

No PEP warranted

CLINICAL PREVENTIVE SERVICES

HIV, Human immunodeficiency virus; PEP, postexposure prophylaxis (see Box 5-7). a For skin exposures, follow-up is indicated only if there is evidence of compromised skin integrity (e.g., dermatitis, abrasion, or open wound). b HIV-Positive, Class 1—asymptomatic HIV infection or known low viral load (e.g., 1,500 RNA copies/mL). HIV-Positive, Class 2—symptomatic HIV infection, acquired immunodeficiency syndrome, acute seroconversion, or known high viral load. If drug resistance is a concern, obtain expert consultation. Initiation of PEP should not be delayed pending expert consultation, and, because expert consultation alone cannot substitute for face-to-face counseling, resources should be available to provide immediate evaluation and follow-up care for all exposures. c Source of unknown HIV status (e.g., deceased source person with no samples available for HIV testing). d Unknown source (e.g., splash from inappropriately disposed blood). e Small volume (i.e., a few drops). f The designation, “consider PEP,” indicates that PEP is optional and should be based on an individualized decision between the exposed person and the treating clinician. g If PEP is offered and taken and the source is later determined to be HIV-negative, PEP should be discontinued. h Large volume (i.e., major blood splash).

Section V

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HIV Postexposure Prophylaxis/Occupational Exposure BOX 5-4

Situations for Which Expert* Consultation for HIV Postexposure Prophylaxis Is Advised

• Delayed (i.e., later than 24-36 hr) exposure report — the interval after which there is no benefit from postexposure prophylaxis (PEP) is undefined • Unknown source (e.g., needle in sharps disposal container or laundry) — decide use of PEP on a case-by-case basis — consider the severity of the exposure and the epidemiologic likelihood of HIV exposure — do not test needles or other sharp instruments for HIV • Resistance of the source virus to antiretroviral agents — influence of drug resistance on transmission risk is unknown — selection of drugs to which the source person’s virus is unlikely to be resistant is recommended, if the source person’s virus is known or suspected to be resistant to 1 of the drugs considered for the PEP regimen — resistance testing of the source person’s virus at the time of the exposure is not recommended • Toxicity of the initial PEP regimen — adverse symptoms, such as nausea and diarrhea are common with PEP — symptoms often can be managed without changing the PEP regimen by prescribing antimotility and/or antiemetic agents — modification of dose intervals (i.e., administering a lower dose of drug more frequently throughout the day, as recommended by the manufacturer), in other situations, might help alleviate symptoms HIV, Human immunodeficiency virus. *Local experts and/or the National Clinicians’ Postexposure Prophylaxis Hotline (PEPline [1-888-448-4911]).

BOX 5-5

Occupational Exposure Management Resources

National Clinicians’ Postexposure Prophylaxis Hotline (PEPline) Run by University of California–San Francisco/San Francisco General Hospital staff; supported by the Health Resources and Services Administration Ryan White CARE Act, HIV/AIDS Bureau, AIDS Education and Training Centers, and CDC. Needlestick! A website to help clinicians manage and document occupational blood and body fluid exposures. Developed and maintained by the University of California, Los Angeles (UCLA), Emergency Medicine Center, UCLA School of Medicine, and funded in party by CDC and the Agency for Healthcare Research and Quality. Hepatitis Hotline Reporting to CDC: Occupationally acquired HIV infections and failures of PEP. Food and Drug Administration Report unusual or severe toxicity to antiretroviral agents.

HIV/AIDS Treatment Information Service

Phone: (888) 448-4911 Internet: http://www.ucsf.edu/hivcntr

Internet: http://www.needlestick.mednet.ucla.edu

Phone: (888) 443-7232 Internet: http://www.cdc.gov/ncidod/diseases/hepatitis/ index.htm Phone: (800) 893-0485 Phone: (800) 332-1088 Address: MedWatch HF-2, FDA 5600 Fishers Lane Rockville, MD 20857 Internet: http://www.fda.gov/medwatch Internet: http://www.hivatis.org

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Occupational Exposure BOX 5-6

465

Management of Occupational Blood Exposures

Provide immediate care to the exposure site: • Wash wounds and skin with soap and water • Flush mucous membranes with water Determine risk associated with exposure: • Type of fluid (e.g., blood, visibly bloody fluid, other potentially infectious fluid or tissue, and concentrated virus) • Type of exposure (i.e., percutaneous injury, mucous membrane or nonintact skin exposure, and bites resulting in blood exposure) Evaluate exposure source: • Assess the risk of infection using available information • Test known sources for HBsAg, anti-HCV, and HIV antibodies (consider using rapid testing) • For unknown sources, assess risk of exposure to HBV, HCV, or HIV infection • Do not test discarded needles or syringes for virus contamination Evaluate the exposed person: • Assess immune status for HBV infection (i.e., by history of hepatitis B vaccination and vaccine response) Give PEP for exposures posing risk of infection transmission: • HBV: See Table 5-13 • HCV: PEP not recommended • HIV: See Tables 5-14 and 5-15 — Initiate PEP as soon as possible, preferably within hours of exposure — Seek expert consultation if viral resistance is suspected — Administer PEP for 4 wk if tolerated Perform follow-up testing and provide counseling: • Advise exposed persons to seek medical evaluation for any acute illness occurring during follow-up HBV exposures • Perform follow-up anti-HBs testing in persons who receive hepatitis B vaccine — Test for anti-HBs 1-2 mo after last dose of vaccine — Anti-HBs response to vaccine cannot be ascertained if HBIG was received in the previous 3-4 mo HCV exposures • Perform baseline and follow-up testing for anti-HCV and alanine aminotransferase (ALT) 4-6 mo after exposures • Perform HCV RNA at 4-6 wk if earlier diagnosis of HCV infection desired • Confirm repeatedly reactive anti-HCV enzyme immunoassays (EIAs) with supplemental tests HIV exposures • Perform HIV-antibody testing for at least 6 mo postexposure (e.g., at baseline, 6 wk, 3 mo, and 6 mo) • Perform HIV antibody testing if illness compatible with an acute retroviral syndrome occurs • Advise exposed persons to use precautions to prevent secondary transmission during the follow-up period • Evaluate exposed persons taking PEP within 72 hr after exposure and monitor for drug toxicity for at least 2 wk HBIG, Hepatitis B immune globulin; HBsAg, hepatitis B surface antigen; HBV, hepatitis B virus; HCV, hepatitis C virus; HIV, human immunodeficiency virus; PEP, postexposure prophylaxis; RNA, ribonucleic acid.

CLINICAL PREVENTIVE SERVICES

Section V

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Occupational Exposure BOX 5-7

Basic and Expanded HIV Postexposure Prophylaxis Regimens

Basic Regimen • Zidovudine (Retrovir; ZDV; AZT) and Lamivudine (Epivir; 3TC); available as Combivir — ZDV: 600 mg per day, in two or three divided doses — 3TC: 150 mg bid Advantages — ZDV is associated with decreased risk of HIV transmission in the CDC case-control study of occupational HIV infection — ZDV has been used more than the other drugs for PEP in HCP — Serious toxicity is rare when used for PEP — Side effects are predictable and manageable with antimotility and antiemetic agents — Can be given as a single tablet (Combivir) bid Disadvantages — Side effects are common and might result in low adherence — Source patient virus might have resistance to this regimen — Potential for delayed toxicity (oncogenic) is unknown Alternative Basic Regimens • Lamivudine (3TC) and Stavudine (Zerit; d4T) — 3TC: 150 mg bid — d4T: 40 mg (if body weight is 60 kg, 30 mg) bid Advantages — Well tolerated in patients with HIV infection, resulting in good adherence — Serious toxicity appears to be rare — Twice daily dosing might improve adherence Disadvantages — Source patient virus might be resistant to this regimen — Potential for delayed toxicity (oncogenic/teratogenic) is unknown • Didanosine (Videx, chewable/dispersable buffered tablet; Videx EC, delayed-release capsule; ddl) and Stavudine (d4T) — ddl: 400 mg (if body weight is 60 kg, 125 mg bid) daily, on an empty stomach — d4T: 40 mg (if body weight is 60 kg, 30 mg bid) bid Advantages — Likely effective against HIV strains from source patients who are taking ZDV and 3TC Disadvantages — ddl is difficult to administer and unpalatable. — Chewable/dispersable buffered tablet formulation of ddl interferes with absorption of some drugs (e.g., quinolone antibiotics, and indinavir). — Serious toxicity (e.g., neuropathy, pancreatitis, or hepatitis) can occur. Fatal and nonfatal pancreatitis has occurred in HIVpositive, treatment-naive patients. Patients taking ddl and d4T should be carefully assessed and closely monitored for pancreatitis, lactic acidosis, and hepatitis. — Side effects are common; anticipate diarrhea and low adherence. — Potential for delayed toxicity (oncogenic/teratogenic) is unknown. Expanded Regimen Basic regimen plus one of the following: • Indinavir (Crixivan; IDV) — 800 mg every 8 hr, on an empty stomach Advantages — Potent HIV inhibitor Disadvantages — Serious toxicity (e.g., nephrolithiasis) can occur; must take 8 glasses of fluid per day — Hyperbilirubinemia common — Requires acid for absorption and cannot be taken simultaneously with ddl in chewable/dispersable buffered tablet formulation (doses must be separated by at least 1 hr) — Concomitant use of astemizole, terfenadine, dihydroergotamine, ergotamine, ergonovine, methylergonovine, rifampin, cisapride, St. John’s Wort, lovastatin, simvastatin, pimozide, midazolam, or triazolam is not recommended — Potential for delayed toxicity (oncogenic/teratogenic) is unknown • Nelfinavir (Viracept; NFV) — 750 mg tid, with meals or snack, or — 1250 mg bid, with meals or snack Advantages — Potent HIV inhibitor — Twice dosing per day might improve adherence Disadvantages — Concomitant use of astemizole, terfenadine, dihydroergotamine, ergotamine, ergonovine, methylergonovine, rifampin, cisapride, St. John’s Wort, lovastatin, simvastatin, pimozide, midazolam, or triazolam is not recommended — Might accelerate the clearance of certain drugs — Potential for delayed toxicity (oncogenic) is unknown

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Basic and Expanded HIV Postexposure Prophylaxis Regimens (Continued)

• Efavirenz (Sustiva; EFV) — 600 mg daily, at bedtime Advantages — Does not require phosphorylation before activation and might be active earlier than other antiretroviral agents (NOTE: this might be only a theoretical advantage of no clinical benefit) — One dose daily might improve adherence Disadvantages — Drug is associated with rash (early onset) that can be severe and might rarely progress to Stevens-Johnson syndrome. — Differentiating between early drug-associated rash and acute seroconversion can be difficult and cause extraordinary concern for the exposed person. — Nervous system side effects (e.g., dizziness, somnolence, insomnia, and/or abnormal dreaming) are common. Severe psychiatric symptoms are possible (dosing before bedtime might minimize these side effects). — Concomitant use of astemizole, cisapride, midazolam, triazolam, ergot derivatives, or St. John’s Wort is not recommended because inhibition of the metabolism of these drugs could create the potential for serious and/or life-threatening adverse events (e.g., cardiac arrhythmias, prolonged sedation, or respiratory depression). — Potential for oncogenic toxicity is unknown. • Abacavir (Ziagen; ABC); available as Trizivir, a combination of ZDV, 3TC, and ABC — 300 mg bid Advantages — Potent HIV inhibitor — Well tolerated in patients with HIV infection Disadvantages — Severe hypersensitivity reactions can occur, usually within the first 6 wk of treatment — Potential for delayed toxicity (oncogenic) is unknown Antiretroviral Agents for Use as PEP Only with Expert Consultation • Retonavir (Norvir; RTV) Disadvantages — Difficult to take (requires dose escalation) — Poor tolerability — Many drug interactions • Saquinavir (Fortovase, soft-gel formulation; SQV) Disadvantages — Bioavailability is relatively poor, even with new formulation • Amprenavir (Agenerase; AMP) Disadvantages — Dosage consists of eight large pills taken bid — Many drug interactions

CLINICAL PREVENTIVE SERVICES

Section V

• Delavirdine (Rescriptor; DLV) Disadvantages — Drug is associated with rash (early onset) that can be severe and progress to Stevens-Johnson syndrome — Many drug interactions • Lopinavir/Ritonavir (Kaletra) — 400/100 mg bid Advantages — Potent HIV inhibitor — Well tolerated in patients with HIV infection Disadvantages — Concomitant use of flecainide, propafenone, astemizole, terfenadine, dihydroergotamine, ergotamine, ergonovine, methylergonovine, rifampin, cisapride, St. John’s Wort, lovastatin, simvastatin, pimozide, midazolam, or triazolam is not recommended because inhibition of the metabolism of these drugs could create the potential for serious and/or life-threatening adverse events (e.g., cardiac arrhythmias, prolonged sedation, or respiratory depression) — May accelerate the clearance of certain drugs — Potential for delayed toxicity (oncogenic) is unknown Antiretroviral Agents Generally Not Recommended for Use as PEP • Nevirapine (Viramune; NVP) — 200 mg daily for 2 wk, then 200 mg bid Disadvantages — Associated with severe hepatotoxicity (including at least one case of liver failure requiring liver transplantation in an exposed person taking PEP) — Associated with rash (early onset) that can be severe and progress to Stevens-Johnson syndrome — Differentiating between early drug-associated rash and acute seroconversion can be difficult and cause extraordinary concern for the exposed person — Concomitant use of St. John’s Wort is not recommended because this might result in suboptimal antiretroviral drug concentrations

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PART C • DISCONTINUATION CONSIDERATIONS TABLE 5-15

Discontinuation Considerations in Preventive Health Screening Hyperlipidemia Colorectal Cancer Breast Cancer Cervical Cancer Prostate Cancer

When patient and physician agree that therapy would not be warranted When early detection is unlikely to prolong life (5-7 more yr) Little evidence to recommend screening in patients 80 yr Until a woman has a life expectancy of 8 to 10 yr At age 65 if previous tests have been consistently normal When patient’s life expectancy is 10 yr or after age 75 for men with average health

Adapted from Takahashi PY et al: Preventive health care in the elderly population: a guide for practicing physicians, Mayo Clin Proc 79:416-427, 2004.

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Systems of Geriatric Care Advance Directives INTRODUCTION The term advance directive refers to designating treatment preferences when one is alert and competent that would go into effect if the person should become unable to make medical decisions on her or his own behalf. Authority for advance directives was relegated to the states by the U.S. Supreme Court in Cruzan vs. Director in 1990 and by Congress in the Patient Self-Determination Act of 1991. Written advance directives generally fall into three categories: living will, power of attorney for health care, and health care proxy. All 50 states and the District of Columbia have statutes that address the creation of one or more form of document. In practice, all states accept duly executed advance directives from other states, and many states have specific statutes noting the acceptance of out-of-state forms. As of 2004, 38 states had Default Surrogate Consent Statutes that address the incapacitated individual with no advance directive and a few states had nonhospital Do Not Resuscitate statutes. A more recent variation is the psychiatric advance directives (PAD). Descriptions of the available advance directives are listed.

ADVANCE DIRECTIVE DOCUMENTS LIVING WILL A living will is a written document that specifies what types of medical treatment are desired should an individual become incapacitated. A living will can be general or very specific. More specific living wills may include information regarding an individual’s desire for such services as analgesia, antibiotics, hydration, feeding, cardiopulmonary resuscitation, and the use of lifesupport equipment. A living will is the most advantageous advance directive for individuals who do not have anyone in their life whom they want to entrust with responsibility for making their health care decisions. The major limitation of a living will is that it is a static document and one cannot anticipate all of the possible scenarios that may be encountered. Many states stipulate that living wills can go into effect only when an individual is terminally ill. Some states further limit the living will by requiring the provision of fluids and nutrition, unless there is a specific statement in the living will of the wish to withhold fluids and nutrition. HEALTH CARE PROXY This is a legal document in which an individual designates another person to make health care decisions if he or she is rendered incapable of making his or her wishes known. The health

care proxy has, in essence, the same rights to request or refuse treatment that the individual would have if capable of making and communicating decisions. It has the major advantage of being flexible, allowing the proxy to make different decisions as conditions change. An alternate proxy is generally included in the execution of the document in case the primary designee is not capable of fulfilling his or her role as proxy (e.g., death, incapacity). These are generally very simple forms to complete. In most states where they exist, they apply whenever the individual is incapacitated, not just when terminally ill. DURABLE POWER OF ATTORNEY FOR HEALTH CARE A number of states have provisions for a document called a durable power of attorney for health care. These are comparable in function to the health care proxy, in which an agent (and alternate agents) are appointed by the individual to make decisions on the individual’s behalf in the event that he or she is no longer capable of doing so. A durable power of attorney for health care generally does not require an attorney to be carried out. It should be pointed out that this is different from a power of attorney that addresses legal and financial issues. A financial power of attorney would allow the designee to make bank transactions, sign Social Security checks, etc., whereas a durable power of attorney for health care applies only to health care decisions. Like a health care proxy, the durable power of attorney for health care is a flexible vehicle and generally applies even when there is no terminal illness. DEFAULT SURROGATE CONSENT STATUTES In the United States, four of every five adults have no advance directive. Thus, it is common for the situation to arise in which an individual is not capable of making his or her own health care decisions and there is no written advance directive. Some states allow for the validity of verbal statements in the absence of written documents. As of July 2004, 38 states had passed Default Surrogate Consent statutes. These statutes establish the right of certain individuals to act as surrogate health care decision makers in the event that a person is incapable of making health care decisions and has not executed an advanced directive. Each state’s statute is different, but most establish a priority order for designation of the surrogate decision maker. The most common prioritization identifies the spouse as the primary surrogate with the following persons in decreasing priority order: adult child, parent, sibling, adult grandchild, and close friend. Some states make provisions for other persons including partners, health professionals, clergy, and beneficiaries, among others.

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Advance Directives DO NOT ATTEMPT RESUSCITATION (DNAR) OR DO NOT RESUSCITATE (DNR) ORDER Some states have a special advance directive that is called a Do Not Attempt Resuscitation (DNAR) or Do Not Resuscitate (DNR) Order. The nonhospital DNR is intended for emergency medical service (EMS) teams, who are usually required to provide every possible life-sustaining service. Even though families expecting a death are advised to call other sources for help when the patient worsens, a moment of uncertainty sometimes results in a 911 call and unwanted measures that prolong death. The nonhospital DNAR or nonhospital DNR Order offers a way for patients to refuse the full resuscitation effort even if EMS is called. It generally must be signed by both the patient and the doctor. PSYCHIATRIC ADVANCE DIRECTIVE A psychiatric advance directive is a document in which persons can describe the kind of mental health treatment they want to receive if they cannot make decisions for themselves in the future. This can include a person’s wishes about medications, electroconvulsive treatment (ECT), or admission to a hospital. As of April 2005, 21 states had passed laws outlining provisions for psychiatric advance directives or health care power of attorneys for mental health treatment.

cussion and not waste time in lengthy discussions with persons who are precontemplative (i.e., not ready to consider the issue). Simply asking the question, “Have you given any thought to how you want to be cared for near the end of life, if you could no longer speak for yourself?” can identify a patient’s level of readiness to deal with the issues. For those who are precontemplative, make a brief statement that this an important issue to consider and provide them a patient information brochure. When the patient is ready (contemplative) then an office-based discussion is warranted. In the likely event that a patient does not complete a written advance directive, but is able to identify a preferred surrogate decision maker, this should be documented in the patient’s chart. Depending on the quality of evidence required in a given state, such documentation may serve as sufficient evidence of a patient’s wishes. If patients have completed an advance directive, a copy should be kept with their other important documents and one copy should be placed in their medical record in their primary care physician’s office. Providing a copy to any surrogate, proxy, or agent is also an important step. It should be noted that in most states an advance directive remains valid until revoked and that it can be revoked at any time. A lawyer is not necessary for the completion of an advance directive.

PRACTICAL CONSIDERATIONS

SUGGESTED READINGS

Encouraging patients to execute advance directives should constitute a routine component of providing health maintenance in primary care. The crisis situation is not the best time to begin discussions on the issue. It is far preferable to institute such discussions when there is ample time to allow for a full discussion of the issues between the patient and his or her family and friends and the physician. However, in a busy office practice there is not sufficient time to devote to an advance directive discussion with each patient. By adopting the readiness to change model (developed for counseling on smoking cessation), one can target the intervention at those who are ready for the dis-

American Bar Association (ABA): The ABA provides a list of state and generally accepted advance directive forms at www.abanet.org/aging/ Basanta WE: Advance directives and life-sustaining treatment: a legal primer, Hematol Oncol Clin North Am 16(6):1381-1396, 2002. Derse AR: Limitation of treatment at the end-of-life: withholding and withdrawal, Clin Geriatr Med 21(1):223-238, 2005. Emanuel LL: Advance directives and advancing age, J Am Geriatr Soc 52(4):641-642, 2004. Westley C, Briggs LA: Using the stages of change model to improve communication about advance care planning, Nurs Forum 39(3):5-12, 2004. AUTHOR: JOHN B. MURPHY, M.D.

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Assisted Living INTRODUCTION Assisted living consists of non–nursing home residential longterm care settings licensed by states. There has been dramatic growth in the number of assisted living facilities in the past 15 years and it is estimated that there are currently as many as 50,000 assisted living facilities operating nationwide. It is further estimated that 1.3 million persons live in assisted living settings. There are a wide variety of designations including board and care, domiciliary care, adult foster care, sheltered housing, residential care, and congregate care. These facilities vary from small family-run settings operating out of a home to large campuses operated by national organizations. Table A1-1 lists names for a variety of different types of facilities and general characteristics of each class of facility.

SERVICES Although the state definitions for assisted living facilities are very diverse and inconsistent, there are some similarities in the services offered in most facilities. Typical services include room and board; 24-hour supervision; housekeeping; maintenance and laundry services; some assistance with activities of daily living (ADL) and instrumental activities of daily living (IADL); limited medical services, often assistance with medications; and structured activities and recreation. Group home settings often serve residents with chronic mental illness or mental retardation and usually have staff-to-resident ratios that are higher than in other assisted living settings.

an individual’s health insurance program or long-term care insurance policy, costs may be reimbursed, but very few individuals have such coverage. Care in an assisted living facility is generally less expensive than in a nursing home but there is also usually less care provided. Regulations for assisted living are likely to be less stringent than in nursing homes, allowing for more efficient (not necessarily better) operations. Costs vary with the residence, room size, and the types of services needed by the residents. Across the nation, daily basic fees range from approximately $15 to $200—generally less than the cost of home health services and nursing home care. A basic assisted living fee may cover all services or there may be additional charges for special services. Most assisted living residences charge month-to-month rates, but a few residences require long-term arrangements.

PHYSICIAN’S ROLE IN ASSISTED LIVING SETTINGS Most patients living in assisted living settings receive their medical care in physician offices and in most states there is no requirement that an assisted living facility have a medical director. The criteria for billing for physician home visits in an assisted living setting are the same as for persons living in private homes (e.g., homebound); however, the CPT codes are different and the payment level is currently lower than for home visits. If there is a medical office established in the assisted living setting the billing is based on outpatient billing codes not nursing home codes.

TARGET POPULATION

SUGGESTED READINGS

The population living in assisted living settings is diverse. Although this population has some overlap with nursing home populations, the assisted living population is less disabled. Common characteristics of the residents are a need for assistance with ADL and IADL, cognitive deficits, and generally less social support in the community.

Eleazer P: Community based care. In Cobbs E, Duthie E, Murphy JB (eds): Geriatric Review Syllabus, ed 5, Malden, MA, 2003, Blackwell, pp 93-100. The National Center for Assisted Living (NCAL): http://www.ncal.org/ consumer/finding.htm Sloane P, Boustani M: Institutional care. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 199-215.

FINANCING Reimbursement for assisted living is largely out-of-pocket, but there are a growing number of states providing support through their Medicaid programs. Depending on the nature of

TABLE A1-1

AUTHOR: JOHN B. MURPHY, M.D.

Assisted Living Facilities and Their Characteristics

Names/Types of Facilities

Characteristics

Personal Care Homes Homes for Adults Board and Care Domiciliary Care Adult Foster Care Senior Group Homes Residential Care Facilities Assisted Living Facilities Adult Congregate Living Continuing Care Retirement Communities Life Care Facilities

Facilities using these names tend to be smaller (fewer than 10 individuals) and less expensive. Many of these are in traditional homes in residential neighborhoods. Shared bathrooms, bedrooms, and living spaces are the norm.

Facilities within this grouping tend to be larger, more expensive, and specifically designed to house the frail elderly or persons with disabilities, with an emphasis on independence and privacy. Most offer private rooms or apartments along with large common areas for activities and meals. These are usually large complexes that offer a variety of options ranging from independent living to skilled-nursing home care. These facilities are specifically designed to provide lifetime care within one community. Facilities within this category tend to be the most expensive option.

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Elderly Driver INTRODUCTION • Motor vehicle accidents among older drivers have been increasing steadily since 1980, particularly for those 85 and older. • Motor vehicle injuries are the leading cause of injury-related death among those age 65 to 74 years and are second only to falls for those 75 to 84 years. • The fatality rate per mile driven is higher for older drivers than any group aside from male drivers under 25. • Older driver involvement in fatal crashes is expected to increase by 155% by 2030, accounting for 54% of the total projected increases in fatal crashes among all drivers. • The responsibility for identifying impaired drivers increasingly falls to medical professionals.

ETIOLOGY • Vision  Safe driving requires good visual acuity and adequate visual fields.  Impairments caused by conditions that increase in prevalence with age (e.g., macular degeneration, glaucoma, cataracts, stroke). • Cognition  Safe driving requires adequate memory, attention, visual processing, and executive functioning.  Impairments caused by conditions that increase in prevalence with age (e.g., stroke, Alzheimer’s dementia). • Physical function  Safe driving requires adequate strength and flexibility as well as intact neurologic function (e.g., proprioception, reaction time).  Impairments caused by conditions that increase in prevalence with increasing age (e.g., rheumatologic conditions, peripheral neuropathies, stroke, deconditioning).

Physical • Visual testing—acuity and visual field testing • Cognitive assessment  Mini-Mental State Exam (memory and attention deficits)  Clock drawing (executive function) • Functional assessment—musculoskeletal and neurologic exams

INTERVENTIONS Reversible impairments: • Stop/taper high-risk medications. • Treat musculoskeletal and rheumatologic conditions. • Physical therapy and exercise for deconditioning. • Cataract removal and refraction. Irreversible impairments: • Possibly unsafe driver  Refer for driver simulator testing (if available).  Refer to agency specializing in road-testing older drivers.  Refer to motor vehicle department for routine road test. • Clearly unsafe driver  Refer to motor vehicle department noting concern.

LEGAL ISSUES Jurisdiction • Dealt with on state level Liability • Some states mandate reporting. • Case law suggests there is risk in not reporting patients who have impairments. • Some states have statutes granting immunity to physicians who report in good faith. • Where statutes do not exist case law suggests physicians are immune if they report in good faith. • At the very least, instruct the patient (or family where appropriate) to stop driving and self-report to the registry of motor vehicles.

DIAGNOSIS History—confirm with family member • Motor vehicle accidents and moving violations • Copilot phenomenon (physically able/cognitively impaired driver, directed by physically impaired/cognitively able passenger) • Past medical conditions  Stroke  Diabetes (vision, peripheral neuropathy)  Macular degeneration, glaucoma, cataracts  Neurologic—stroke, TIA, dementia, seizures, neuropathy • Medications—sedative-hypnotics, narcotic analgesics, sedating antidepressants, antihistamines, and alcohol

SUGGESTED READINGS American Medical Association: Physician’s guide to assessing and counseling older drivers, June 2003: www.ama-assn.org/go/olderdrivers Freund B et al: Clock drawing and driving cars, J Gen Intern Med 20:240-244, 2005. Grabowski D, Campbell AB, Morrisey MA: Elderly licensure laws and motor vehicle fatalities, JAMA 291:2840-2846, 2004. Hogan DB: Which older patients are competent to drive? Can Fam Physician 51:362-368, 2005. AUTHOR: JOHN B. MURPHY, M.D.

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Home Care INTRODUCTION When physicians prescribe home care services for Medicare beneficiaries, they must certify that the patient is (1) homebound; (2) in need of intermittent skilled nursing care or physical, speech, or occupational therapy; and (3) under the physician’s ongoing care. By signing a standard authorization form approved for home care services by the Centers for Medicare & Medicaid Services (CMS), the physician verifies that the patient has met the three eligibility criteria and that the physician will review the home care plan periodically but no less than every 2 months and recertify the patient if appropriate. Even though physicians may not have firsthand knowledge that the home care services they prescribe are appropriate and necessary, prescribers of home care need to know that the federal government’s position is that “when a physician signs a Medicare certification form, there is an implied representation that all the rules are complied with.” In fact, the official form for the home care plan contains a statement that “misrepresentation, falsification or information concealment may be subject to fine, imprisonment or civil penalty.” The eligibility criteria for home care are stringent because the intent of the Medicare program is generally to cover acute care rather than long-term care or preventive care. However, the clinical reality for many patients is that their chronic conditions exacerbate and improve over time, causing them to shift in and out of home care eligibility. These changes in condition complicate the physician’s role in determining patients’ eligibility for home care services covered by Medicare. This topic discusses the role of the physician in authorizing and monitoring home care services given CMS regulations.

THE HOMEBOUND CRITERIA If the federal Medicare program defined home confinement literally, patients who visit doctors in their offices while receiving home care would not be, strictly speaking, confined to their home. Fortunately, CMS does not define home confinement literally. Currently, patients need not be bedridden, but “there should exist a normal inability to leave home and leaving their homes would require a considerable and taxing effort.” For practical purposes, Medicare considers patients homebound if they lack the ability to leave their place of residence independently; however, such patients may leave their home with the aid of assistive devices (e.g., canes, crutches, walkers, wheelchairs), special transportation (e.g., ambulance, van), or another person (e.g., family member). Still, not all patients who use canes are eligible for home care. Medicare also expects that absences from the home be infrequent or relatively short in duration (e.g., a trip to the hairdresser or to church), and that in most instances absences from the home be for the purpose of medical treatment. Patients may also be considered homebound if leaving the home is medically contraindicated. The criteria for home confinement are rather subjective. Therefore, how physicians, home care agencies, patients, and CMS define home confinement may differ. For example, in a review of claims for home care in New York and Texas, CMS found that 40% of the claims were improperly billed, with the majority of improper billings resulting from patients failing to meet home confinement criteria. Box A1-1 lists clinical situations that would qualify patients for meeting the Medicare criteria for home confinement.

SKILLED SERVICES REQUIREMENT A frail person who is homebound is not considered eligible for home care unless criteria for intermittent skilled care are also met. The criteria for skilled care are even more ambiguous than those for home confinement. Skilled care is care that must be provided by a registered nurse, or physical, occupational, or speech therapist. However, just because a service is provided by

one of these health professionals does not necessarily mean the service is a skilled service. To be considered a skilled service, a nurse must be required to provide it because of “its complexity” and “the patient condition.” The service must also meet “accepted standards of medical and nursing practice.” A diagnosis alone is rarely adequate documentation of the need for skilled services. Rather, the relation between a patient’s diagnosis, symptoms, and functional status must justify the complexity of services. In addition, the need for skilled services must be intermittent, meaning that the services are required less frequently than 7 days per week, but at least once every 60 days. CMS provides a list of examples that do and do not meet this requirement (Table A1-2). The documentation in the medical record should describe the patient’s condition and the complexity of required services and include an assessment of the risk of complications or deterioration should such skilled services become unavailable. These same principles of medical documentation apply for physical, occupational, and speech therapy.

THE PHYSICIAN AS GATEKEEPER The home care gatekeeping role may be appropriate for primary care physicians in many cases. However, nurses, therapists, or social workers may sometimes be better suited to determine home care eligibility because of the nature of the patient’s condition or because, unlike physicians, they routinely make house calls. Without firsthand knowledge of the patient’s homebound status, physicians should not certify the patient in a perfunctory manner as confined to home. Physicians should take the time to assess the patient’s functional status and need for skilled services before prescribing home care services. In some cases, the physician who prescribes home care has no doubt that the patient meets the Medicare criteria for home confinement. Examples include a patient with a dense hemiparesis, a patient with advanced dementia, or a patient who recently underwent major surgery. These patients are incapable of leaving their home independently or have a medical contraindication to do so; if they have a need for intermittent skilled care, they meet the Medicare criteria for in-home care. For many patients, however, physicians may not know whether they are eligible to be certified for home care. For example, a diabetic patient with a foot ulcer who is capable of driving her car may benefit greatly from home care for wound

BOX A1-1 Examples of Homebound Cases

According to Medicare Criteria 1. Restricted mobility from a disease process such as unsteady gait, draining wounds, or pain. 2. Poor cardiac reserve, shortness of breath, or activity intolerance as a result of an unstable or exacerbated disease process. 3. Bed- or wheelchair-bound patients who require physical assistance to move any distance. 4. Patients who require caregiver help with assistive devices such as a cane, walker, wheelchair, or other special device to leave home. 5. A tracheostomy, abdominal drains, colostomy, Foley catheter, or nasogastric tube that restricts ambulation. 6. Psychotic ideation, confusion, or impaired mental status that restricts functional abilities outside the home. 7. Fluctuating blood pressures or blood sugar levels that may cause syncope. 8. Patients who cannot ambulate stairs or uneven surfaces without assistance of caregiver. 9. Postoperative patients for whom the physician has restricted patient activity. 10. Patients who are legally blind or cannot drive.

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Home Care management and diabetic teaching by a visiting nurse, but her physician knows or should know that she is able to drive to her doctor’s office to receive the wound care and teaching. She is not eligible for home care services. Patients who need physical therapy or daily monitoring of vital signs following hospital discharge may not meet the eligibility criteria for home confinement if they can travel independently to their doctor’s office or to a rehabilitation facility. Yet, physicians prescribe home care in such situations without much afterthought because wound care, diabetic teaching, gait training, and blood pressure checks are performed by nurses or physical or occupational therapists. In each of these cases, for the patient to receive outpatient nursing care, usually available only through home care agencies, the officebased primary care physician who does not employ a nurse will prescribe home care and certify that patient as home confined, which may be a misrepresentation of the patient’s status. Thus physicians are sometimes placed in conflicting roles as advocates for their patients and as gatekeepers for CMS eligibility criteria for home care. This conflict is even more pronounced when patients have chronic conditions such as congestive heart failure or chronic obstructive pulmonary disease and only meet criteria for skilled services during episodes of exacerbation. Yet, in-home services for those conditions have been shown to reduce exacerbations and hospitalizations. Physicians should always pay sufficient attention to home care certification and plan-of-care forms completed by home care agency staff and mailed to physicians for signature. Physi-

TABLE A1-2

cians should not rely solely on the recertification plan-of-care forms sent to them as the forms may not contain adequate information about the patient’s functional status and condition. Physicians should demand that these forms document not only patients’ current needs for skilled care, but also the reasons for their homebound status. Copies of these forms should be entered into the medical record and used to justify the in-home skilled services provided. Physicians also should not allow long periods to go by (e.g., 6 months for a stable patient) without seeing patients who receive home care services; this may require house calls for some patients. Patients whose chronic diseases are unstable (e.g., those with cardiopulmonary conditions) may require more frequent updates, not only to provide good care, but also to verify that they remain eligible for needed home care services. Inability to carry out activities of daily life, cognitive impairment, and urinary incontinence correlate with the need of in-home services. Given that an assessment of functional status is an integral component of geriatric care, the medical record can and should contain up-to-date functional assessments that can be used to justify patients’ home confinement and need for skilled services.

SUGGESTED READING Wachtel TJ, Gifford DR: Eligibility for home care certification: the doctor’s job? J Gen Intern Med 13:705-709, 1998. AUTHOR: TOM J. WACHTEL, M.D.

Clinical Vignettes Related to Medicare Home Care Eligibility Criteria

Vignette

Home Confined

Skilled Care Need

Meets Medicare Eligibility Criteria

Patient with unsteady gait who requires assistance for ambulation and whose blood pressure is 190/110 Patient with a dense hemiparesis who is bed- or chairbound and who has a pressure ulcer Patient with severe peripheral neuropathy and blindness who is wheelchair dependent for mobility and whose diabetes is well controlled with insulin Patient with advanced Alzheimer’s dementia, incontinent of urine, and living in his daughter’s home. No other medical problem. Patient with a draining venous ulcer who is able to walk and drive her car independently Patient with severe emphysema and cor pulmonale who is ambulatory and stable on home oxygen therapy and medication

Yes

Yes

Yes

Yes

Yes

Yes

Yes

No

No

Yes

No

No

No

Yes

No

No

No

No

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Hospice INTRODUCTION Hospice is a term that is used for a number of entities and activities. Hospice is a benefit offered by Medicare, most Medicaid programs, and many private insurers. Hospice refers to palliative care provided near the end of life and to a physical place where end of life care is provided. For the purpose of this discussion the focus will be on the Medicare benefit and the services provided under the Medicare hospice benefit. In 1983, Congress enacted the hospice benefit within the Medicare program. Because of an aging of the population, a greater understanding of the place of palliative care in the continuum of care, and growth in the number and quality of hospice organizations, there has been considerable growth in the number of persons enrolled in hospice since the inception of the benefit. That growth has been most dramatic in the past 10 years, which has seen annual hospice admissions rise from 200,000 to almost 1,000,000. Under Medicare, hospice is a program of care delivered by a Medicare-approved hospice. Reasonable and necessary medical and support services for the management of a terminal illness are furnished under a plan-of-care established by the beneficiary’s attending physician and the hospice team. Over 90% of services are provided in the beneficiaries’ homes. Respite and general inpatient services are also provided when socially or medically necessary. Although most physicians think of hospice and palliation with cancer diagnoses, many other conditions are also appropriate for hospice care. National data show that 50.5% of hospice patients die from cancer. The top five noncancer diagnoses are end-stage heart disease, dementia, lung disease, end-stage kidney disease, and end-stage liver disease. The common thread is that their physicians feel the patients are unlikely to survive more than 6 months if their disease runs its natural course. National data also suggest that the referral to hospice is often much later in the course of an illness than is appropriate.

ELIGIBILITY Hospice care is available under Medicare in the following circumstances: • The patient is eligible for Medicare Hospital Insurance (Part A). • The patient’s doctor and the hospice medical director certify that the patient is terminally ill with 6 months or less to live if the disease runs its expected course. • The patient signs a statement choosing hospice care instead of standard Medicare benefits for the terminal illness. • The patient receives care from a Medicare-approved hospice program. Hospice care is a benefit under the hospital insurance program. To be eligible to elect hospice care under Medicare, an individual must be entitled to Part A of Medicare and be certified as being terminally ill. An individual is considered terminally ill if the medical prognosis is that the individual’s life expectancy is 6 months or less if the illness runs its normal course. Section §1814(a)(7) of the Social Security Act specifies that certification of terminal illness for hospice benefits shall be based on the clinical judgment of the hospice physician and the individual’s attending physician (if he or she has one) or the medical director regarding the normal course of the individual’s illness. Once a patient enters a hospice program the first certification is for 90 days. The first recertification is also for 90 days, and all subsequent recertifications are for 60 days. Recertifications require only a single physician signature. A physician can discharge a patient from a hospice program, and patients can revoke or transfer hospice programs at any time. Predicting of life expectancy is not always exact. The fact that a beneficiary lives longer than expected in itself is not cause to terminate benefits. Once the initial election is processed, the beneficiary remains in hospice status until death or until the patient elects to termi-

nate hospice. While on hospice an individual must waive all rights to Medicare payments for the duration of the election of hospice care for the following services: • Hospice care provided by a hospice other than the hospice designated by the individual (unless provided under arrangements made by the designated hospice) • Any Medicare services that are related to the treatment of the terminal condition for which hospice care was elected or a related condition or services that are equivalent to hospice care, except for services provided by:  The designated hospice (either directly or under arrangement)  Another hospice under arrangements made by the designated hospice  The individual’s attending physician, if that physician is not an employee of the designated hospice or receiving compensation from the hospice for those services Medicare services for a condition completely unrelated to the terminal condition for which hospice was elected remain available to the patient if he or she is eligible for such care.

COVERED SERVICES Once enrolled in a hospice, the hospice is financially responsible for all diagnostic and therapeutic services related to the terminal disease. This may include palliative treatments, medications, therapies, and any respite or medically related admissions. For conditions totally unrelated to the hospice diagnosis, a patient can be admitted to a hospital, treated at an outpatient facility, or cared for at a physician’s office under traditional Medicare. Medicare covers: • Physicians’ services • Nursing care (intermittent with 24-hour on call) • Medical appliances and supplies related to the terminal illness • Outpatient drugs for symptom management and pain relief • Short-term acute inpatient care, including respite care, • Home health aide and homemaker services • Physical therapy, occupational therapy, and speech/language pathology services • Medical social services • Counseling, including dietary and spiritual counseling • Bereavement services It should be noted that these services are provided as part of symptom control or to enable the individual to maintain activities of daily living and basic functional skills and not as part of a curative care plan. There are four levels of hospice care • Routine home care • Continuous home care • Respite care • General inpatient care Routine home care is provided as medically necessary and involves at least nursing care and social service with overall direction by a hospice medical director. The frequency and intensity of service depend on medical needs, but usually increase over time. Continuous home care is 8 or more hours daily, provided to a patient in crisis, with at least 50% of that care performed by licensed nurses. Respite care involves admission to an institution (skilled nursing facility [SNF] or other) for up to 5 days when needed to allow the family a temporary break from their caregiving duties. General inpatient care (GIP) is provided by the hospice under contract with a facility (SNF or other) and is for short-term provision of intensive, aggressive management of acute symptoms, which cannot be provided in another setting. In addition, GIP may be provided when a beneficiary’s home support system has acutely deteriorated, and short-term placement is required while arranging a more permanent placement option. Nursing home residents are eligible for hospice within their long-term care facility under the same eligibility criteria described previously.

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Hospice PHYSICIAN’S ROLE IN HOSPICE The hospice medical director provides administrative and general oversight of the program. The patient’s hospice attending physician (who can also be the medical director) is reimbursed for all hands-on care. If the attending physician is salaried by the hospice, the hospice bills Part A. If the attending physician is not employed by the hospice, the Part B carrier is billed using regular evaluation and management (E & M) codes with the modifier GV (not employed by hospice). The attending physician is listed in the Medicare common working file. Physicians covering for the attending physician should use locum tenens rules (modifier GV with Q5 or Q6 as appropriate). For non-hospice-related conditions and services, the physician uses regular E & M codes plus the modifier GW (service not related to the hospice patient’s terminal condition). The primary medical attending physician continues to bill Medicare directly for services and may bill under comprehensive procedural technology (CPT) code 99375 for phone management of the hospice patient if it exceeds 30 minutes per month. Consultations (e.g., with an oncologist) related to the terminal illness must be paid for by the hospice and require prior authorization by the hospice pro-

gram. Unauthorized testing or treatment for the terminal illness is not covered by Medicare and becomes the patient’s responsibility. Treatment of disease unrelated to the terminal illness may be independently covered by Medicare, including reimbursement of attending and consulting physician fees. This is more likely to occur for the patient who enrolls in hospice early in the course of his or her illness.

SUGGESTED READINGS Fried T et al: Older persons’ preferences for site of terminal care, Ann Intern Med 131:109-112, 1999. Lorenz K, Lynn J: Care of the dying patient. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 323-334. Pinderhughes S, Morrison R: Palliative care. In Cobbs E, Duthie E, Murphy JB (eds): Geriatric Review Syllabus, ed 5, Malden, MA, 2003, Blackwell, pp 105-115. Potash J, Horst P: Palliative care at the end of life. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 229-242. AUTHOR: JOHN B. MURPHY, M.D.

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House Calls and Domiciliary Visits INTRODUCTION A growing number of people in the U.S. are homebound. Some live at home; others live in congregate housing arrangements such as elderly housing, independent living, assisting living, boarding houses, and group homes. House calls refer to the provision of physician services to patients in their home. House calls are generally provided in lieu of office-based medical care for selected patients. This care may be delivered as part of an interdisciplinary care team, as in home health care following a hospitalization, but more commonly as ongoing care to patients who are homebound. House calls can also be exceptionally helpful in providing the physician with a better understanding of the patient’s home situation. This type of “diagnostic house call” can provide information about caregiver burnout, elder mistreatment, use of medications that the primary physician is not aware of, and alcohol abuse. It can also be helpful when the treatment plan is unexpectedly not effective. For a house call program to be effective and financially viable it is imperative that the program be well planned. Current regulations allow for reimbursement to physicians, nurse practitioners, and physician assistants, but involvement of other health care services (e.g., laboratory and visiting nurses services) is essential. Choosing the right patients is also of paramount importance. Strictly speaking, no one is homebound. There is always a way to bring a homebound person to the hospital or to a physician’s office. However, except in the case of a medical emergency, transportation by ambulance is not covered by most health insurance plans, and ambulance trips cost several hundred dollars. The definition of homebound that applies to the Centers for Medicare & Medicaid Services (CMS) eligibility criteria for formal home care (see Home Care in Appendix 1) does not concern house calls by physicians/practitioners. Many physicians who perform house calls use stricter criteria because their offices are better equipped to provide care than patients’ homes. Therefore, a patient who is unable to leave his or her home unassisted because of dementia but has a relative who can provide transportation to the doctor’s office may not be a suitable candidate for house calls, although the CMS criterion for homebound is met. For practical purposes, a homebound person can be defined as someone who cannot leave the home, transiently (e.g., after surgery) or permanently, using personal or other resources readily available in the community (e.g., friends, relatives, taxi service, senior citizens’ transportation, public transportation). Such a person should receive health services at home. Some programs are designed to provide multidisciplinary geriatric assessments with patients’ homes. There are no Medicare CPT reimbursement codes to pay providers for such service; therefore, the provision of care by a single provider is described.

THE HOUSE CALL Most first visits at home are scheduled well in advance. Therefore, it is almost always possible for the informal caregiver (most frequently the patient’s daughter) to be present during the visit. Even when a family provides care in shifts, arrangement can be made for a key member of the informal caregiving team to be present. When the patient’s condition requires substantial nursing services (e.g., a patient with an active pressure ulcer), every effort should be made for the visiting nurse to be present during the visit; this will enable the whole team to discuss the plan of care. Taking a history in the patient’s home is no different in its content than a history taken in the office, but because it is done on the patient’s turf, often over coffee and muffins, the interaction tends to be less formal. Permission should be requested to

inspect the living quarters. What does the home look like? Is it clean? Is it orderly? Can the patient get around, go to the kitchen, go to the bathroom? Is the environment safe? Are there loose rugs, nightlights, rails in the bathroom? An office visit, no matter how comprehensive, cannot provide a complete understanding of the patient’s daily routine. The cabinet or drawer that contains the medications should be inspected. Outdated and discontinued medications should be discarded immediately. The refrigerator and food cupboards should also be checked, especially when the homebound patient depends on others for his or her food supply. Aside from appearance, what does the home smell like? Incontinence may be immediately detected. Many elderly patients are reluctant to report incontinence in the office. It is more difficult to hide during a house call. Observing the interaction between caregivers and patients is also a precious source of information. In the office, patients and families are generally on their best behavior and outbursts are unlikely to occur. In the home setting, people are less inhibited, even in the presence of the physician. Therefore, they are more likely to display their usual pattern of interaction. Moreover, the request for a house call is often placed at a time of crisis. Most often, the crisis is not a new medical problem but rather an exhausted caregiver who needs support or respite. Although the goal of a house call will certainly vary, like any physician-patient interaction, the content of a home visit should include a number of staple items (Table A1-3). Because most candidates for home care are frail or disabled, a functional assessment should always be performed. This includes gathering information on physical function, such as activities of daily living (ADL), instrumental activities of daily living (IADL), mobility, and continence; social and role function, such as visits by friends and relatives, and interest in sexual activity; and mental function, such as cognition and affect. Unlike the office setting, where such assessment must be done formally by asking specific questions, much of this information can be collected by simple observation during a home visit. A more complete checklist describing the relation between the patient and the home environment is presented in Fig. A1-1. Asking homebound elderly patients to discuss ethical issues such as care intensity should be a routine practice. In the hospital, a common and important question is whether to resuscitate. At home the equivalent issue is whether to hospitalize. Whatever decision is made, it is entered into the patient’s record. Equally important is the question of surrogate decision makers. Competent patients are routinely requested to think about designating someone who will have durable power of attorney for medical decisions. This is also entered into the medical record. Homebound patients and their informal caregivers should be given a list of community resources. The physical exam is often less complete at home than in the office or at the hospital. Although technically feasible, a pelvic exam for cancer screening may not be indicated in an elderly patient with Alzheimer’s dementia. Although a bimanual exam is possible, expecting to visualize a cervix with an elderly patient lying in a regular bed is unreasonable. Most patients who receive care at home do not expect the same comprehensive services routinely provided to ambulatory patients; nonetheless, it is best to be explicit and explain to patients the medical goals. Blood and urine tests and an occasional ECG can be obtained as readily in the home as in the office (or community-based laboratories). Portable x-rays can be obtained at great expense and poor quality. Typically, a situation that calls for x-rays or other diagnostic procedures requires evaluation in a hospital.

LOGISTICS AND TIME MANAGEMENT The logistics of house calls may explain why many physicians, busy with their office and hospital work, find house calls an inefficient use of their time. There is obviously some validity to

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House Calls and Domiciliary Visits this argument. However, steps can be taken to reduce travel time. The physician with a substantial number of homebound patients can arrange to make routine visits to a cluster of patients who live in the same neighborhood. The fact that older members of our society tend to congregate in elderly housing makes clustering easy to accomplish. A photocopy of a map illustrating the location of the patient’s home is placed in front of the patient’s chart. Except for first encounters, two to four house calls can be scheduled per hour when several visits are arranged in the same apartment building or neighborhood. In addition, routine house calls can be made to replace idle time caused by cancellations in the office. This can actually improve the efficiency of a geriatric practice (e.g., during bad weather). House calls for urgent problems (e.g., a fever) may be impossible to plan in a physician’s schedule. Urgent visits can be made at the end of the day on the way home from work. However, it should be made clear to homebound patients that emergencies cannot always be dealt with in the home. The willingness to make house calls should not eliminate use of traditional emergency services when necessary. Physicians and practitioners who make house calls should specify the conditions under which they will provide this service. For example: • Patients are eligible for the house calls program if they live within a 15-minute drive and are unable to leave their home. • Scheduled visits are made every 3 to 4 months. A half-day of a physician’s schedule per month may be reserved for these visits, and patients are also seen during last-minute unfilled cancellations in the ambulatory practice schedule. Unscheduled visits are made for more urgent problems at the request of the patient, a family member, or a home care nurse. • On enrollment, patients and their caregivers should be given an information sheet stating that although efforts will be made to see patients with acute problems, the practice cannot guarantee the ability to make emergency visits and cannot provide home visits at night or during weekends. Physicians and practitioners who provide house calls should have a tool kit (“doctor’s bag”) with the following equipment: Basic Supplies • Gloves • Sphygmomanometer • Oto-ophthalmoscope • Stethoscope • Thermometer

TABLE A1-3

• Lubricating jelly • Stool guaiac cards and developer • Toenail clippers • Ear syringe and/or cerumen curette • Tongue depressors • Hand disinfectant/wash • Scale (in patient’s home) • Prescription pad • Patient chart with directions • Local street map • Cell phone • Dictaphone Additional Advisable Supplies • Voice amplifier • Foley catheter • Wound debridement kit • Wound dressing materials • Specimen cups • Portable scale pulse oximeter • Lancets and Glucometer with alcohol pads, 2x2 gauze, and bandages • Urine dipsticks • Peak flow meter Supplies for Special Circumstances • Punch biopsy kit • Excisional biopsy kit • Suture/staple removal kit The chronic conditions that physicians can expect to manage during house calls are the same as those seen in the office for an age-matched population with a bias toward conditions that impair mobility such as dementia, post stroke, severe arthritis, and severe CHF and COPD. Acute illnesses include the following: • Infections, mostly respiratory and urinary • Exacerbation of CHF or COPD • Syncope • Falls • Delirium • Wounds (pressure ulcers, venous ulcers, arterial ulcers, diabetic ulcers) • Gastroenteritis and abdominal pain • Acute musculoskeletal problems (injuries, arthralgia) • Acute and chronic pain • Failure to thrive • End-of-life care

Problems to Address during a Geriatric Home Visit

Safety Household risks (e.g., loose rugs, nightlights, bathroom rails) Security Appliances, fire alarm Dirt, dust, humidity Access to telephone or other means of calling for help Psychosocial Situation Mental status Affect (rule out depression) Support (family, friends, rule out loneliness) Financial resources Ethical Issues Intensity of care (especially terminal care) Decision to (not to) hospitalize Surrogate decision maker (durable power of attorney) or living will Functional Status Activities of daily living (ADL) Instrumental activities of daily living (IADL) Mobility Vision and hearing assessment

Continence Urine and stool Nutrition Availability of food Ability to prepare and eat meals Alcoholism Medical Problems Tertiary prevention (diagnose, treat, and monitor chronic diseases) Primary prevention (vaccination, hygiene) Diagnose and treat acute illness Prognosis Skin care, foot care Primary Care Access and availability of care Coordination of team of health care professionals Continuity of care

( ) Bed bound

( ) Chair bound

Ability to reach and use phone

Ability to manage TV

Ability to transfer on/off chairs

Ability to maneuver through room

C. Living/Family Room

NOTE: Lighting, obstacles

Access mail

Open door

Unlock door

Landing

Walkway

Driveway

B. Entrance

NOTE: Stair covering and railing, smoke alarms, emergency exits, extension cords

Can patient escape safely (e.g., fire)

Through doorways

Thresholds

Uneven terrain

Ramp

Stairs

Elevator

A. General Obstacle Negotiation

I

I

I

A

A

A

D

D

D

NA

NA

NA

( ) Ambulatory with walker

( ) Stairs to upstairs

( ) Rarely/never alone

8. Function in the home (I, independent; A, assistance required; D, dependent; NA, not applicable)

7. IADL

6. ADL

( ) Ambulatory with care

( ) Stairs to main floor

( ) Alone part of the day

( ) Ambulatory without care

5. Mobility

( ) None (or ramp)

4. Stairs

( ) Completely alone

Name(s), Relation(s) ______________________ ________________________________________

3. Who lives at the residence:

( ) Apartment

( ) House

2. Previous living situation

( ) Apartment

( ) House

1. Type of dwelling

I

I

I

I

A

A

A

A

D

D

D

D

NA

NA

NA

NA

FIGURE A1-1 Checklist for the traditional home evaluation. (From Ferri F, Fretwell MD, Wachtel TJ: Practical Guide to Care of the Geriatric Patient, ed 2, St. Louis, 1997, Mosby.)

NOTE: What medications are found; outdated, no longer prescribed?

Ability to open containers

Ability to follow instructions

Ability to identify medication

G. Medications

NOTE: Lighting, obstacles

Ability to use closets and drawers

Ability to reach light switch

Ability to reach phone

Ability to transfer in/out of bed

Ability to maneuver to and through room

F. Bedroom

shower bench; tub, shower, or toilet rails; hot water temperature

NOTE: Lighting (also between bedroom and bathroom); obstacles; nonskid surface (tub and shower); tub and

Ability to reach sink, faucet, grooming supplies

Ability to transfer in/out of tub or shower

Ability to transfer on/off toilet

Ability to maneuver to and through room

E. Bathroom

NOTE: Lighting, floors, reachability of utensils/dishes/food; the quantity and quality of the food

Ability to prepare meals

Ability to use appliances (refrigerator, stove, sink)

Ability to reach shelves/cabinet

Ability to use table and chair

Ability to maneuver through room

D. Kitchen

NOTE: Lighting, floors, emergency numbers by phone

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House Calls and Domiciliary Visits PAYMENT FOR HOUSE CALLS • Home visits (includes senior housing and independent living)  New patient CPT codes: 99341, 99342, 99343, 99344, 99345. The documentation requirements for these codes are similar to office-based new patient evaluation and management (E&M).  Established patient CPT codes: 99347, 99348, 99349, 99350; also similar to office visit codes (Table A1-4). • Domicilliary visits (include assisted living and group homes)  New patient CPT codes: 99321, 99222, 99323. The documentation requirement for 99321 is a problem-focused history and examination and low-complexity decision making. For 99322, it is expanded problem-focused history and examination and moderate-complexity decision making. For 99323, it is detailed history and high-complexity decision making.  Established patient CPT codes 99331, 99332, and 99333 with the same documentation requirements as for followup nursing home patients.

• In addition, CMS pays physicians for overseeing the work done by home care agencies. The CPT codes for these services are:  G0180 certification for home care  G0179 recertification for home care  G0181 care plan overnight for home care (requires at least 30 minutes per month and ability to document the time spent)  G0182 care plan oversight for hospice (same time requirements)

SUGGESTED READINGS American Academy of Home Care Physicians: Executive summary, public policy statement, 2005. Boling P, Abbey L, Keenan J: Home care. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 217-228. Eleazer P: Community based care. In Cobbs E, Duthie E, Murphy JB (eds): Geriatric Review Syllabus, ed 5, Malden, MA, 2003, Blackwell. Unwin B, Jerant A: The home visit, Am Fam Physician 60:1481-1488, 1999. AUTHORS: TOM J. WACHTEL, M.D., and JOHN B. MURPHY, M.D.

CPT Codes and Approximate 2004 Medicare Allowable Charges

TABLE A1-4

Code

Visit Type

99341 99342 99343 99344 99345 99347 99348 99349 99350

New patient, low severity New patient, moderate severity New patient, moderate to high severity New patient, high severity New patient, unstable Established patient, minor Established patient, low to moderate severity Established patient, moderate to high severity Established patient, high severity

2004 Medicare Allowable Charges (before geographic adjustments) $57.87 $84.38 $123.21 $161.67 $200.13 $44.43 $75.42 $116.86 $169.89

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Life Expectancy INTRODUCTION 1. Definitions and Concepts a. Life expectancy is defined as the average length of life expected for a population of individuals of a given age. Life expectancy, not otherwise specified, is calculated from birth. Age-specific life expectancy is calculated for a group of individuals of a specific age. b. U.S. life expectancies based on the 2000 census are presented in Table A1-5. c. The calculation of life expectancy is based on the age configuration of the population at the time that a census is taken; therefore life expectancies are calculated as if an individual were able to live every year of his or her life in the present state of population mortality. This, of course, is not true; the younger a person is, the more likely to benefit from advances in community health and medical progress or be penalized by changes in morbidity prevalence (e.g., obesity) or other social calamities such as war. d. As people age, the differences between men and women or between African Americans and Caucasians attenuate. e. Maximum life span is defined as the length of the longestlived members of a species. The longest recorded human life is 122 years (a French woman). Current research suggests that average human life span in the absence of premature death from disease or trauma approximates 85 years with a standard deviation of 4 to 5 years. Biotechnology may alter these statistics in the future. f. Life span is species-specific and is believed to be genetically determined; an almost perfect correlation exists between maximum cell doublings in tissue culture for a given species and the life span for that species. Therefore, it should not be surprising to find longevity running in certain families. However, the mechanism of aging

(senescence) has not been identified; and although theories abound (e.g., accumulation of oxygen free radicals, growth hormone deficit), most interventions to correct them (e.g., antioxidants, growth hormone) have not been proven to be effective fountains of youth. The only promising means to increase longevity to date are food restriction (works in rats) and lowering body temperature (works in hamsters). g. Current thinking attributes the limit on life span to a progressive decline in organ reserve that begins for humans at age 20 to 30. Reserve function is needed to respond to lifetime stress. When reserve function has declined to about 20% above basal levels, routine daily perturbations cannot be overcome, homeostasis cannot be maintained, and death will occur with minor illnesses or trauma that would be trivial for vigorous individuals. 2. Compression of mortality (and morbidity) and the rectangularization of the population survival curve (Fig. A1-2) a. Although human life span has not varied during the past millenary, human life expectancy has increased dramatically. For example, life expectancy from birth in the United States in 1900 was 47 years; and in 2002 it was 77.3 years. This gain can be attributed to improvement in infant mortality and primary preventive measures such as sanitation and immunization. Progress in and increased access to medical care can also take some credit for the gains in life expectancy. b. The combination of a finite limit on life span and an increase in life expectancy results in more individuals achieving their maximum life potential of 85  5 years in the population. This trend is demonstrated graphically by a narrowing bell-shaped curve of the number of deaths (Fig. A1-2, B).

Life Expectancy by Race, Sex, and Age: U.S. 2002*

TABLE A1-5

ALL RACES Age 0. 1. 5. 10 . 15 . 20 . 25 . 30 . 35 . 40 . 45 . 50 . 55 . 60 . 65 . 70 . 75 . 80 . 85 . 90 . 95 . 100 .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

. . . . . . . . . . . . . . . . . . . . . .

WHITE

BLACK

Total

Male

Female

Total

Male

Female

Total

Male

Female

77.3 76.8 72.9 67.9 63.0 58.2 53.5 48.7 44.0 39.3 34.8 30.3 26.1 22.0 18.2 14.7 11.5 8.8 6.5 4.8 3.6 2.7

74.5 74.1 70.2 65.3 60.3 55.6 51.0 46.3 41.6 37.0 32.6 28.3 24.1 20.2 16.6 13.2 10.3 7.8 5.7 4.2 3.2 2.5

79.9 79.4 75.4 70.5 65.5 60.7 55.8 51.0 46.1 41.4 36.7 32.2 27.7 23.5 19.5 15.8 12.4 9.4 6.9 5.0 3.7 2.8

77.7 77.2 73.3 68.3 63.4 58.6 53.8 49.0 44.3 39.6 35.0 30.5 26.2 22.1 18.2 14.7 11.5 8.7 6.4 4.7 3.4 2.4

75.1 74.6 70.7 65.7 60.8 56.1 51.4 46.7 42.0 37.4 32.9 28.5 24.3 20.3 16.6 13.3 10.3 7.7 5.7 4.1 3.0 2.3

80.3 79.7 75.8 70.8 65.9 61.0 56.1 51.2 46.4 41.6 36.9 32.4 27.9 23.6 19.5 15.8 12.3 9.3 6.8 4.9 3.5 2.5

72.3 72.4 68.5 63.6 58.7 53.9 49.3 44.7 40.1 35.6 31.3 27.3 23.4 19.9 16.6 13.5 10.9 8.6 6.6 5.1 3.9 3.0

68.8 68.8 65.0 60.1 55.2 50.5 46.0 41.6 37.1 32.8 28.5 24.6 21.0 17.6 14.6 11.8 9.5 7.5 5.8 4.5 3.6 2.9

75.6 75.6 71.7 66.8 61.8 57.0 52.1 47.4 42.7 38.1 33.7 29.5 25.4 21.6 18.0 14.7 11.7 9.2 7.0 5.3 4.0 3.0

Source: National Vital Statistics Reports, Vol. 53, No. 6, November 10, 2004. *Race categories are consistent with the 1977 Office of Management and Budget guidelines.

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Life Expectancy

482

3. Future Trends a. The rectangularization of population survival, the compression of morbidity, and the compression of senescence are predicated on the hypothesis of a finite limit of organ reserve (Fig. A1-3, A). As stated by Fries, “As the organ reserve decreases so does the ability to restore homeostasis, and eventually even the smallest perturbation prevents homeostasis to be restored. The inevitable result is natu-

100

80

80 Number of deaths

100

Survivors (%)

A

ral death, even without disease” or after a short acute illness (e.g., pneumonia). b. Alternately, progress in medical technology (e.g., artificial organs) could conceivably halt the trend toward rectangularization and simply move the entire survival curve to the right (Fig. A1-3, B). c. The secular trend toward rectangularization of population survival curves is not inconsistent with a (transient) con-

60 40

B

40 10

10 0

60

20

40

60

80

0

100

20

40

60

80

100

Age (yr) Age (yr) FIGURE A1-2 A, Proportion of survivors in the population (Y axis). B, Number of deaths per year in each 1-year age bracket. Solid line, present; dotted line, future. (From Ferri F, Fretwell MD, Wachtel TJ: Practical Guide to Care of the Geriatric Patient, ed 2, St. Louis, 1997, Mosby.)

80

80 Survivors (%)

100

Survivors (%)

60 40 10 0

60

B

40 10

20

40

60

80

0

100

20

40

Age (yr)

60

80

100

Age (yr) 100 80

FIGURE A1-3 A, Rectangularization of population survival curve as a result of disease prevention and mortality compression (solid line: present population survival curve, broken line: conceptual future survival curve). B, Prolongation of life expectancy without mortality/morbidity compression in a model where clinical disease is not prevented but only postponed and treated aggressively and successfully in older people (solid line: present population survival curve, broken line: conceptual future survival curve). C, Change in a population survival curve where oldest segment of population is experiencing greatest decline in mortality (solid line: present population survival curve, broken line: conceptual future survival curve). (From Ferri F, Fretwell MD, Wachtel TJ: Practical Guide to Care of the Geriatric Patient, ed 2, St. Louis, 1997, Mosby.)

Survivors (%)

A

100

60

C

40 10 0

20

40

60 Age (yr)

80

100

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Life Expectancy temporary widening of the period of morbidity and mortality associated with recent data that indicate that the elderly citizens of our population are enjoying the greatest decline in mortality (Fig. A1-3, C). d. Possible reasons for the recent downturn in cardiovascular disease and related deaths can be attributed to reduced dietary saturated fat and cholesterol levels, positive developments in medical care for coronary heart disease and stroke, better treatment of hypertension, and improved access to health care for the elderly since 1965 because of the Medicare program. 4. Age-adjusted life expectancy is often underestimated for older individuals because clinicians are familiar with life expectancy figures from birth only. An 80-year-old woman has a life expectancy of 9.5 years: should she be in good general baseline health, an acute life-threatening, but reversible, illness should be treated as aggressively as in a younger person barring specific reasons not to (including patient preference).

Increasing life expectancy is the reason that the relentless issue of social security solvency keeps resurfacing on the political agenda. Indeed because it is funded by taxing working people, the declining ratio of working to retired people in the population can only lead to an increasing tax burden, a decrease in the benefit (payment), raising retirement age, or some combination of those (see Fig. A1-4). The same problem applies to the funding of the Medicare system.

SUGGESTED READINGS Fries JF: Aging, natural death, and the compression of morbidity, N Engl J Med 303:130-135, 1980. Olshansky SJ, Carnes BA, Cassel C: In search of Methuselah: estimating the upper limits to human longevity, Science 150:634-640, 1990. Olshansky SR et al: A potential decline in life expectancy in the United States in the 21st century, N Engl J Med 352:1138-1145, 2005. Wachtel TJ: The connection between health promotion and health care costs. Does it matter? Am J Med 94:451-454, 1993. AUTHOR: TOM J. WACHTEL, M.D.

80 70

Age

60

Years in Retirement Working Years

50 40 30

20 1900 1910 1920 1930 1940 1950 1960 1970 1980 1990 2000 Year FIGURE A1-4 Impact of increasing life expectancy in the population ratio of time in retirement and time at work.

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Medicare and Medicaid

484

INTRODUCTION In 1965, the Social Security Act established Medicare and Medicaid, Titles XVIII and XIX, respectively. When the Medicare program was enacted in 1966 it was a responsibility of the Social Security Administration (SSA), whereas the Medicaid program was administered by the Social and Rehabilitation Service (SRS). SSA and SRS were agencies in the Department of Health, Education, and Welfare (HEW). In 1977, the Health Care Financing Administration (HCFA) was created under HEW to effectively coordinate the Medicare and Medicaid programs. In 1980, HEW was divided into the Department of Education and the Department of Health and Human Services (HHS). In 2001, HCFA was renamed the Centers for Medicare & Medicaid Services (CMS).

MEDICARE Medicare is a health insurance program for Americans age 65 or older. Currently, Medicare provides coverage to approximately 40 million persons. Certain people younger than age 65 can qualify for Medicare, including some individuals who have disabilities and all individuals who have end-stage kidney disease. The program helps with the cost of health care, but it does not cover all medical expenses or the cost of most long-term care. Medicare is financed by payroll taxes paid by workers and employers, general tax dollars, and the monthly premiums of beneficiaries. Medicare has several parts, including hospital insurance (Medicare Part A) and medical insurance (Medicare Part B). These two parts of Medicare help pay for different kinds of health care costs. The sources of Medicare funding are different for each part. Part A is funded by the 1.45% payroll tax. Part B funding comes from two sources, 75% from general federal revenues and 25% from beneficiary premiums. In 2003 the source of funding for Medicare was 55% payroll taxes, 28% general tax revenues, 9% premiums, and 8% from interest and taxes on SS benefits. MEDICARE PART A Medicare Part A helps pay for inpatient care in a hospital or skilled nursing facility (SNF) following a hospital stay, some home health care, and hospice care. The specific components of Medicare Part A coverage are listed in Table A1-6. Although for most beneficiaries there is no premium for Part A, there are deductibles, co-pays, and limitations on the duration of coverage. The deductibles and co-pays for 2005 are listed in Table A1-7 along with the limitations in duration of coverage. The coverage

TABLE A1-6 • • • •

Medicare Part A Coverage Benefits

Hospital Inpatient Mental Health Skilled Nursing Facility Home Health Care

• Hospice Care • Blood

TABLE A1-7

Semiprivate room, meals, general nursing, and other hospital services and supplies. Limited to 190 days in a lifetime. Semiprivate room, meals, skilled nursing and rehabilitative services (after a related 3-day inpatient hospital stay). Limited to reasonable and necessary part-time skilled nursing care and home health aide services as well as physical therapy, occupational therapy, and speech-language therapy. For people with a terminal illness, includes drugs for symptom control and pain relief, medical and support services from a Medicare-approved hospice. Blood transfused in a hospital or skilled nursing facility during a covered stay.

Medicare Part A Deductibles, Co-pays, and Limitations

• Hospital Coverage (2005)

• Skilled Nursing Facility

• Hospice

is based on a benefit period, which is defined as 60 days out of the hospital. MEDICARE PART B Medical insurance (also called Medicare Part B) helps pay for doctors’ services and many other medical services and supplies that are not covered by Part A. The specific components of Medicare Part B coverage are listed in Table A1-8. Unlike Medicare Part A, Part B requires the beneficiary to pay a monthly premium that will increase annually as the national cost of Medicare Part B increases. The monthly premiums, co-pays, and deductibles for Medicare Part B are listed in Table A1-9. Some of the items not covered by Medicare A or B are listed in Table A1-10. MEDICARE PART C (MEDICARE MANAGED CARE PLANS) Medicare Managed Care plans were previously known as Medicare Plus Choice and now as Medicare Advantage plans. They are health insurance programs offered by Medicare-qualified organizations to Medicare-eligible individuals, in lieu of Original Medicare plan coverage. The health plans receive 95% of the age-adjusted per capita cost (AAPCC) for Medicare beneficiaries in the region. The plans are at risk and can make a profit or loss. At a minimum they must provide the same benefits offered under Original Medicare plan coverage but may offer benefits beyond traditional Medicare. The programs generally “manage” the care offered to beneficiaries to minimize unnecessary services and diminish costs to the organization. They come in a variety of forms. Examples of Medicare Advantage plans include: • HMO plans (health maintenance organization plans), which offer no coverage for providers or facilities outside the HMO network and almost always require a network primary care physician referral to access a network specialist. • POS plans (point of service plans) offer a network of preferred providers, like HMO plans, but also provide reduced benefits for providers or facilities outside the HMO network. • Regionally expanded preferred provider organization (PPO) plans are similar to POS plans but have broader geographic access to network providers in a larger service area, and reduced benefits outside the PPO network. • PSO plans, otherwise known as provider-sponsored organizations, are similar to the POS plans but are usually organized with physicians that practice in a regional or community hospital.

$912 for a hospital stay of 1–60 days. $228 per day for days 61–90 of a hospital stay. $456 per day for days 91–150 of a hospital stay. (Lifetime Reserve Days do not recur per benefit period.) All costs for each day beyond 150 days (all costs beyond 90 days once Lifetime Reserve Days exhausted). Nothing for the first 20 days. $114 per day for days 21–100. All costs beyond the 100th day in the benefit period. Nothing for Medicare-approved services. 20% of the Medicare-approved amount for durable medical equipment.

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Medicare and Medicaid MEDICARE PART D Beginning January 1, 2006, beneficiaries will choose one of at least two private plans or Medicare Advantage plans that offers drug coverage in their locale. The initial Medicare Part D drug benefit enrollment period begins November 15, 2005, and lasts until May 15, 2006. The Medicare Part D drug benefit is optional. However, Medicare beneficiaries who do not enroll in Part D during the initial enrollment period and who decide to enroll in Part D later will pay a higher premium. There are no provisions in the Medicare drug benefit law to contain drug costs. In fact, the legislation prohibits Medicare from using its purchasing power to negotiate lower drug prices for beneficiaries. The specifics of the Medicare Drug benefit are depicted in Fig. A1-5. MEDIGAP POLICIES A Medigap policy is a health insurance policy sold by a private insurance company to fill “gaps” in Original Medicare plan coverage. The Omnibus Budget Reconciliation Act (OBRA) of 1990 required that Medigap plans be standardized in as many as 10 different benefit packages offering varying levels of supplemental coverage. All policies sold since July 1992 (except in three exempted states) have conformed to 1 of these 10 standardized benefit packages, known as plans A through J. In 1999, about 10.7 million Medicare beneficiaries (more than one fourth of all beneficiaries) had a Medigap policy to help cover Medicare’s cost-sharing requirements as well as some benefits not covered by Medicare parts A or B.

ture jointly funded by the federal and state governments to assist states in furnishing medical assistance to eligible needy persons. Medicaid is the largest source of funding for medical and health-related services for America’s poorest people. Within broad national guidelines established by federal statutes, regulations, and policies, each state (1) establishes its own eligibility standards; (2) determines the type, amount, duration, and scope of services; (3) sets the rate of payment for services; and (4) administers its own program. Medicaid policies for eligibility, services, and payment are complex and vary considerably, even among states of similar size or geographic proximity. Thus, a person who is eligible for Medicaid in one state may not be eligible in another state, and the services provided by one state may differ considerably in amount, duration, or scope from services provided in a similar or neighboring state. In addition, state legislatures may change Medicaid eligibility, services, or reimbursement during the year. The Balanced Budget Act (BBA) included a state option known as Programs of All-inclusive Care for the Elderly (PACE). PACE provides an alternative to institutional care for persons aged 55 or older who require a nursing facility level of care. The PACE team offers and manages all health, medical, and social services and mobilizes other services as needed to provide preventative, rehabilitative, curative, and supportive care.

Premiums, Deductibles, and Co-pays for Medicare Part B

TABLE A1-9

MEDICAID Title XIX of the Social Security Act is a federal/state entitlement program that pays for medical assistance for certain individuals and families with low incomes and resources. This program, known as Medicaid, became law in 1965 as a cooperative ven-

• Part B premium • Deductible • Co-pays

TABLE A1-8

Components of Medicare Part B

Coverage • Medical and Other Services:  Doctors’ services (not routine physical exams*)  Outpatient medical and surgical services and supplies  Diagnostic tests  Ambulatory surgery center facility fees for approved procedures  Durable medical equipment  Outpatient mental health care  Outpatient occupational and physical therapy, including speechlanguage therapy • Outpatient Hospital Services • Blood Transfusions • Preventive Services  Bone mass measurements  Cardiovascular screening (starting January 1, 2005, fasting lipid profile)  Colorectal cancer screening  Diabetes services  Glaucoma testing  Pap test and pelvic examination (if indicated)  Prostate cancer screening  Screening mammograms  Vaccinations—influenza and Pneumovax  “Welcome to Medicare” physical examination includes □ Measurement of height, weight, and blood pressure □ An ECG □ Education and counseling. *Except a one-time comprehensive assessment at enrollment.

$78.20 per month in 2005* (set at 25% of Medicare Part B cost). $110 (in 2005) deductible (once per calendar year). 20% of the Medicare-approved amount after the deductible. 20% for all outpatient physical, occupational, and speech-language therapy services. 50% for most outpatient mental health care.

*Beginning in 2006 the Medicare Part B Premium will be means tested and the annual premium for beneficiaries will rise with income. under $80,000 ($160,000 for couples), 25% of Part B costs. between $80,000 and $100,000, 35% of Part B costs. between $100,000 and $150,000, 50% of Part B costs. between $150,000 and $200,000, 65% of Part B costs. over $200,000, 80% of Part B costs ($3,000/year in 2005 dollars).

TABLE A1-10

Items not covered by Medicare

Part A or B • • • • • • • • • • • • • •

Acupuncture Dental care and dentures Cosmetic surgery Custodial care at home or in a nursing home Health care while traveling outside of the United States Hearing aids and hearing exams for the purpose of fitting a hearing aid Hearing exams (screening) Long-term care, such as custodial care in a nursing home Orthopedic shoes Outpatient prescription drugs (see Medicare D) Routine foot care Routine eye care and most eyeglasses Routine or yearly physical exams (except the “Welcome to Medicare” PE) Screening tests (except those listed above under preventive services)

485

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Medicare and Medicaid Long-term care is an important provision of Medicaid that will be increasingly utilized as our nation’s population ages. The Medicaid program paid for over 41% of the total cost of care for persons using nursing facility or home health services in 2001. National data for 2001 show that Medicaid payments for nursing facility services totaled $37.2 billion for more than 1.7 million beneficiaries of these services. With the percentage of our population who are elderly or disabled increasing faster than that of the younger groups, the need for long-term care is expected to increase. Medicare beneficiaries who have low incomes and limited resources may also receive help from the Medicaid program. For such persons who are eligible for full Medicaid coverage, the Medicare health care coverage is supplemented by services that are available under their state’s Medicaid program, according to eligibility category. These additional services may include, for example, nursing facility care beyond the 100-day limit covered by Medicare, prescription drugs, eyeglasses, and hearing aids.

For persons enrolled in both programs, any services that are covered by Medicare are paid for by the Medicare program before any payments are made by the Medicaid program, because Medicaid is always the “payer of last resort.” Starting January 2006, the new Medicare prescription drug benefit will provide drug coverage for Medicare beneficiaries, including those who also receive coverage from Medicaid. In addition, individuals eligible for both Medicare and Medicaid will also receive the low-income subsidy for both the Medicare drug plan premium and assistance with cost sharing for prescriptions. Medicaid will no longer provide drug benefits for Medicare beneficiaries.

SUGGESTED READING Centers for Medicare & Medicaid Services: http://www.cms.hhs.gov/ AUTHOR: JOHN B. MURPHY, M.D.

Drug Benefit

5%

Benefit $5,100.00 Total Drug Spending ($3,600 Out-of-Pocket)

Individual Cost-Sharing

Median Drug Spending for 2006: $1891

$2,250 25%

75%

Median Drug Spending for 2006: $1891

$250 FIGURE A1-5 Medicare Part D drug benefit, as of January 1, 2006.

* $

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Nursing Home Care, Introduction and General Issues NURSING HOMES Nursing homes have evolved from the Alms Houses of a century ago into highly regulated institutions providing care to primarily older individuals with profound medical, psychiatric, and social disabilities. In the last 10-20 years, the degree of regulation and the level of impairment of nursing home residents have increased dramatically. In the United States, there are roughly 17,000 nursing homes with 1.8 million beds that care for 1.6 million persons receiving custodial care (nursing facility, NF) or skilled care (skilled nursing facility, SNF). Most nursing homes provide both NF and SNF levels of care. Two thirds of the nation’s nursing homes are for-profit facilities, and nearly 55% of all homes are part of large regional or national chains. Roughly 25% of nursing homes are voluntary not-for-profit facilities and about 10% are government affiliated. The average nursing home is about 100 beds and fewer than 10% have more than 200 beds.

NURSING HOME STAFFING Nursing home staff include the following groups: nursing, social work, administration, therapists (may be full time or provided by an outside agency and include physical therapy, occupational therapy, and speech pathology), activities staff, dietary, housekeeping, and maintenance. Nursing universally accounts for the majority of the staff and minimally trained nurses aides make up the bulk of the nursing staff. Relatively few licensed nurses are present to conduct assessments, distribute medications, supervise nurses aides, communicate with physicians, and administer treatments. Only a small percentage of nursing homes, typically the largest nursing homes, have employed physicians. A 1996 Institute of Medicine report recommended increased staffing levels as an important step to increase quality of care in nursing homes, but the financial resources to achieve these increases are lacking. The recruitment and retention of nursing staff is a major challenge, with turnover rates for licensed nurses as high as 50% per year and still higher for nursing assistants.

NURSING HOME RESIDENTS The lifetime risk of a nursing home stay is currently estimated to be 43%. The risk of being in a nursing home increases with age: 1.5% for Americans age 65-74 and 20% for those 85 and older. The average length of stay in a nursing home is about 2 years, with 25% having stays of 3 months or less, 50% with stays of 1 year or longer, and 20% staying as long as 5 years. As a group, nursing home residents are increasingly disabled. Only 2.8% of nursing home residents are independent in all activities of daily living (ADL) and 83% require assistance with three or more ADL. Dementia is one of the most frequent conditions of nursing home residents, estimated at a prevalence of 50%-70%. Behavioral problems are common (33%), as are communication problems that are seen in more than 50% of residents. Depression is also very common, estimated at a prevalence of 20%. Most nursing home residents suffer from many chronic illnesses. One half of nursing home residents are 85 years or older and less than 10% are younger than 65. Most nursing home residents are women (72%), and although 89% are white, black persons are actually at higher risk of a nursing home stay at earlier ages than white persons. Additional factors associated with admission to a nursing home are low income, poor family supports (particularly the lack of a daughter), and cognitive impairments.

FINANCING OF LONG-TERM CARE Nursing home expenditures currently exceed $100 billion per year and are expected to top $150 billion by 2007. Public expenditures account for roughly two thirds of all nursing home spending; with Medicaid expenditures double that of Medicare. Medicare covers only short-term posthospital stays whereas Medicaid pays for custodial care for persons who have exhausted their personal finances. The remaining one third of

nursing home costs are out-of-pocket expenditures, with a very small amount being covered by long-term care insurance. Nearly one third of persons who enter a nursing home paying privately will “spend down” and end up on Medicaid. Almost all facilities charge private-pay residents more than Medicaid, often twice as much. Thus, the nursing home with a high self-pay payer mix will be more financially able to provide higher quality services and is more likely to be profitable. The cost of physician payments for nursing home residents is covered by Medicare Part B and, when applicable, Medicaid. The typical nursing home base cost per year is in excess of $60,000, although there is considerable geographic variation.

QUALITY ASSURANCE Beginning with the Federal Nursing Home Reform Act of 1987 (OBRA 87), there have been a number of initiatives to enhance quality. The federal government has established minimum staffing guidelines, training guidelines, basic resident rights, and comprehensive mandatory resident assessments. The standardized resident assessment is known as the minimum data set (MDS) and focuses on clinical issues of quality of care. The MDS will trigger resident assessment protocols (RAPs) for identified problems. Quality indicators (QIs) are a relatively new initiative to enhance quality of care in nursing homes. Each is described below. MINIMUM DATA SET (MDS) The minimum data set quantifies current or potential problems in medical, psychologic, or functional domains. The MDS is completed for residents admitted for a non-Medicare stay within 14 days of admission, annually, and when a significant change in resident status occurs. In addition, an abbreviated MDS is conducted quarterly. For patients admitted for a stay covered by Medicare Part A, the MDS is completed more frequently. The MDS is completed by the nursing home staff and should be available for the physician to review, but unfortunately many physicians do not avail themselves of these data. When the MDS is completed, certain item responses identify or trigger one of a number of RAPs. RESIDENT ASSESSMENT PROTOCOLS (RAPS) The RAPs that are triggered by the MDS include issues common to nursing home populations and are listed below. The RAPs highlight areas that need attention and guide the care team through further assessment and intervention (care plan). • Delirium • Cognitive loss and dementia • Visual functioning • Activities of daily living (ADL) • Functional rehabilitation potential • Urinary incontinence and indwelling catheters • Psychosocial well-being • Mood • Behavioral symptoms • Activities • Falls • Nutritional status • Feeding tubes • Dehydration and fluid maintenance • Dental care • Pressure ulcers • Psychotropic drug use • Physical restraints QUALITY INDICATORS (QIS) QIs are designed to highlight existing or potential problems as a means to guide each facility’s QA (quality assurance) program. QIs can cover a number of domains that include quality indicators. Examples are listed in Table A1-11. QIs may be compared among facilities that are similar, as a way to benchmark standards. Although each nursing home must conduct a QA program that represents all major disciplines, the design of such programs is not standardized.

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Nursing Home Care, Introduction and General Issues ROLE OF THE PHYSICIAN

SUGGESTED READINGS

Medical care of nursing home residents offers unique challenges and demands exemplary clinical skills. The physician practicing in a nursing home must embrace interdisciplinary care and be sensitive to ethical issues. Nursing home care will include care that is preventative, curative, or palliative. The care requires a biopsychosocial approach. Table A1-12 lists the responsibilities of the physician practicing in the nursing home setting. These responsibilities reflect standards of care as well as specific regulations. The regulations encompass several domains, each of which corresponds to a regulatory code known as a tag number. The domains address physician services, resident assessment, resident rights, and quality of care. Tables A1-13 and A1-14 list suggested time management guidelines for efficient nursing home practice and the CPT codes for physician billing in the nursing home.

Donius M. Comprehensive assessment in an institutional setting. In Ostreweil D, Brummel-Smith, Beck J (eds): Comprehensive Geriatric Assessment, New York, 2000, McGraw-Hill, pp 225-251. Katz P: Nursing home care. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 197-209. Katz P, Karuza J: Nursing home care. In Cobbs E, Duthie E, Murphy JB (eds): Geriatric Review Syllabus, ed 5, Malden, MA, 2003, Blackwell. Sloane P, Boustani M: Institutional care. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 199-216.

TABLE A1-11 Examples of Nursing Home Quality Indicators Domain

Quality Indicator

Accidents

Incidence of new fractures Incidence (prevalence) of falls Prevalence of weight loss Prevalence of tube feeding Prevalence of dehydration Prevalence of antipsychotic use without appropriate reason Prevalence of sedative/hypnotic use Prevalence of pressure ulcers, Stage 1-4

Nutrition

Psychotropic drug use

Skin care

TABLE A1-12 Physician Responsibilities in the Nursing Home 1. Comprehensively assess each resident, assist in care plan development and coordination of all aspects of care. (Tag 272, 279) 2. Periodically review the care plan and assure that the goals and objectives for each care plan are rational and functionally relevant. (Tag 272, 279, F 250, F 309) 3. Implement treatments and services to enhance or maintain physical and psychologic function and to avoid accidents. (Tag F 502-512, F 310, F 311, F 323, F 324) 4. Physically attend to each resident in a manner consistent with state and federal guidelines (visit every 30 days for the first 90 days following admission, and at least every 60 days thereafter) while assuring that the appropriate diagnostic tests are performed and followed up in a timely manner. (Tag F 387, F 500-512) 5. Respond in a timely fashion to a resident’s change in function or condition. (Tag F 157) 6. Inform residents of their health status and optimize ability for residents to exercise self-determination. (Tag F 151, 152, and 154). 7. Determine each resident’s decision-making capacity while establishing advance directives. (Tag F151 and F152) 8. Assure that residents are free from unnecessary drugs by periodic review of drug regimens and consultant pharmacist recommendations. (Tag F 329-331, F 428 and F 429)

AUTHOR: JOHN B. MURPHY, M.D.

TABLE A1-13

Time Management Guidelines

• Limit practice to only a few facilities. • Employ a nurse practitioner or physician’s assistant who can manage routine care and serve as a liaison between yourself and nursing staff and families. • Establish regular days for rounding in a particular nursing home. • Always cluster routine visits, avoid seeing a single patient on one visit. • Develop protocols for common problems. • Address advance directives with resident and family soon after admission. • Establish strong relationships with nursing staff and nursing home administrative staff. • Conduct rounds with the floor nurse to ensure acquisition of key information and to make sure plans are carried out appropriately. • Train staff to limit after-hours calls to urgent medical problems and establish a system for conveying nonurgent information (e.g., regularly scheduled call).

TABLE A1-14 CPT Codes for Nursing Facility Services (New 2006 Codes) COMPREHENSIVE NURSING FACILITY ASSESSMENTS, ADMISSIONS AND RE-ADMISSIONS 99304 99305 99306

Low complexity (e.g., simple annual assessment) Moderate complexity (e.g., complex annual assessment or simple admit/readmit) High complexity (e.g., complex admission or readmit) SUBSEQUENT NURSING FACILITY CARE, ESTABLISHED PATIENT

99307 99308 99309 99310 99315 99316 99318

Low (e.g., stable, recertification) Moderate (e.g., change in treatment, new complaint, not responding) Serious medical event or several unstable medical conditions High (e.g., major medical event) Nursing facility discharge management, less than 30 minutes Nursing facility discharge management, more than 30 minutes Annual comprehensive assessment

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Nursing Home Visits, Documentation, and Physician Payment INTRODUCTION

DOCUMENTATION

It is generally expected that patients will be cared for by their attending physician within the nursing home. The availability of consultants/specialists in nursing homes is variable and nursing home residents often need to be transported to specialist offices or hospitals for consultations. In the hospital setting, daily visits by the attending physician are appropriate, expected, and reimbursed by third-party payers. Ambulatory patients are seen episodically by their primary care physician and have some degree of control over the frequency of their office visits. Nursing home patients do not usually need to be seen on a daily basis, but they should be visited by their attending physician according to some schedule. The Centers for Medicare & Medicaid Services (CMS) has created such a schedule to assure “reasonable” physician visitation in the long-term care setting. The Medicare Career Manual describes payment of physician’s visits to residents of nursing facilities and nursing facilities in section 15509.1: • Nursing home visits must comply with federal regulations (42 CFR 483.40).

• The volume of documentation should not be the primary influence on which a specific level of service is billed. Documentation should simply support the level of service reported. • Assessment documentation  See sample form, which can be used for admissions, readmissions, and annual comprehensive visits (Fig. A1-6). • Follow-up regulatory visits  At the minimum, these notes must include: 1. Date 2. Reason for visit (e.g., follow-up for diabetes and CHF) 3. Relevant interval history (may refer to nurses notes and telephone calls) 4. Physical examination 5. Interval diagnostic test result acknowledgment 6. Assessment or impression or diagnosis 7. Plan of care 8. Legible identity (signature) of writer The 30-day/60-day regulatory visit should address the medical problems that are under current active management. For example, blood sugar results should be mentioned in diabetic patients, blood pressure should be mentioned in hypertensive patients, mental function or behaviors should be mentioned in patients treated with psychoactive medication. Because most nursing home residents have multiple comorbid conditions, SOAP notes may not be the most efficient way to document care. • Sick visits should be problem focused and would typically resemble daily progress notes in a hospital record.  Chief complaint/reason for visit  Symptoms  Examination  Diagnosis  Treatment SOAP notes may be appropriate for such visits. • What evaluation of management (E & M) CPT code to use? The national frequency of NH visit codes is presented in Table A1-15. Individual physician/practitioner coding profiles should generally reflect these statistics. All E & M codes have performance and documentation requirements. Each code has standards that pertain to: • History • Examination • Medical decision making CPT codes 99304, 99305, 99306, and 99318 are typically straightforward because of the comprehensive nature of these visits. Documentation requirements for subsequent visit CPT codes 99307, 99308, 99309, and 99310 are complex. The same rules apply to regulatory visits and sick visits, which complicates the task of selecting the “correct” CPT code.

RESIDENT ASSESSMENTS Medicare Part B pays for visits necessary to perform all Medicare-required assessments. Physicians should use the CPT codes for comprehensive nursing facility assessments (9930499306 and 99318) to report these evaluation and management services. Comprehensive assessments must be coupled with the creation of a minimum data set (MDS) and resulting resident assessment protocols (RAPs). This occurs at the time of admission, readmission, once-a-year routinely, and whenever a change in condition requires a new MDS (e.g., a stroke).

SUBSEQUENT VISITS Subsequent visits are classified as routine (or regulatory) and sick visits. These visits and all other medically necessary visits for the diagnosis or treatment of illness or injury or to improve the functioning of a body part are covered under Medicare Part B. Physicians should use CPT codes for subsequent nursing facility care (99307-99310) when reporting evaluation and management services that do not involve resident assessments. Medicare pays for visits required to monitor and evaluate residents once every 30 days for the first 90 days after admission and once every 60 days thereafter. Medical necessity of a service is a key criterion for payment. Medical necessity is assumed to exist when regulatory visits are performed. The schedule for those visits is determined by the date of admission rather than the previous visit. The timing can be difficult to comply with because auditors allow 10 days before or after the scheduled date is “consistent with the requirement,” but a visit “offschedule” or sick visit does not reset the clock. The physician or practitioner determines the necessity for sick visits. A patient with pneumonia who is managed in a nursing home can be seen appropriately every day while unstable. A patient with onychomycosis, treated with an antifungal agent, does not need daily visits to monitor progress. Providing unnecessary services is called Medicare abuse (fraud is billing for a service that was not provided).

DISCHARGE SERVICES Assessment visits and subsequent regulatory or sick visits require face-to-face contact between the patient and the practitioner/physician. Discharge services (CPT codes 99315 and 99316) may not require face-to-face contact on the day of discharge (controversial): the billing date is the discharge date, but the service is typically provided over several days; it includes discharge forms, prescriptions, phone calls, a discharge summary, etc. It is a time-based service: 99315, 30 minutes or less; 99316, longer than 30 minutes. These codes can be used if the reason for discharge is death.

TABLE A1-15

NH Visits—2000 CMS.gov* Code

%

99301 99302 99303 99311 99312 99313 99315 99316

2.0 2.9 4.8 37.4 41.4 10.3 1.0 0.3

*These codes are now obsolete.

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Nursing Home Visits, Documentation, and Physician Payment COMPREHENSIVE HISTORY & PHYSICAL EXAMINATION ATTENDING PHYSICIAN ____________________________________________________ DATE ____________________________ NAME: ____________________________________________________________________ AGE _____________________________ REASON FOR ADMISSION:__________________________________________________________________________________________ ____________________________________________________________________________________________________________________ ____________________________________________________________________________________________________________________ ____________________________________________________________________________________________________________________ PAST MEDICAL HISTORY: __________________________________________________________________________________________ ____________________________________________________________________________________________________________________ ____________________________________________________________________________________________________________________ ____________________________________________________________________________________________________________________ Social History/Habits: Marital Status: Living Situation: Smoking: Alcohol: Other: Family History: ____________________________________________________________________________________________________ Functional Status: Basic ADLs Ambulation: Independent Dependent __ unassisted __ with walker Transfers _____________ _____________ __ with cane __ non-ambulatory Feeding _____________ _____________ Urine: Bathing _____________ _____________ continent __ incontinent __ Dressing _____________ _____________ Stool: Grooming _____________ _____________ continent __ incontinent __ Review of Symptoms: Informant: Patient __ Staff __ Family __ NO YES NL ABN Headache □ □ Hearing □ □ Chest pain □ □ Vision □ □ Abdominal Pain □ □ Urination □ □ Pain in Limbs □ □ Bowel □ □ Other Pain □ □ Sleep □ □ SOB □ □ Appetite □ □ Dizziness □ □ Energy □ □ Fall □ □ Memory □ □ EXAM: Vital signs: BP _____________ P _____________ Weight _____________ Ears: Hearing _____________ Lungs _____________ TM _____________ Heart _____________ Eyes: Vision (L) _____________ (R) _____________ Abdomen _____________ ROM _____________ Fields _____________ Rectal: Pupils _____________ Prostate _____________ Nose: _____________ Mucosa _____________ Throat: _____________ Stool _____________ Neck: LN _____________ Ortho: _____________ Thyroid _____________ Neuro: Carotids _____________ Motor _____________ Breasts & Axillae: _____________ Reflexes _____________ Extremities: Gait _____________ Pulses _____________ Mental Status: _____________ Edema _____________ Affect: _____________ Skin: _____________ Laboratory Data

Radiology ECG ECHO

Goals of Admission: Rehabilitation/postacute care □ Long-term care □ Advance Directives: ________________________________________________________________________________________________ Assessment and Plan:

Attending Physician:_________________________________________________________

Date _____________________________

FIGURE A1-6 Comprehensive History & Physical Examination

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Nursing Home Visits, Documentation, and Physician Payment ASSESSMENT/COMPREHENSIVE VISITS The work and documentation presented on the form included in this chapter will generally satisfy the requirement for these CPT codes: • 99318 should be used for an overall annual review of the patient’s medical problems. This service is not required, but it is desirable and allowed by Medicare. It should occur contemporaneously with a new MDS but Medicare does not specify how closely the two activities should coincide. • 99306 is used for most new admissions and some readmissions to a long-term care facility (skilled or nonskilled). • 99305 is used for occasional moderately complex admissions and many (skilled or nonskilled) readmissions following hospital stays that result in relatively little change in the patient’s condition or plan of care (e.g., noncardiac chest pain or a urinary infection). • 99304 is used for (unusual) simple admissions or re-admission (e.g., a physically healthy person with dementia only). Admission and readmission visits should occur as soon as possible after the admission to the nursing home. The condition of the patient may or may not require a visit within 24-48 hours of admission, but waiting longer than 1 week is not advisable. Medicare allows up to 30 days to complete the admission. A sick visit may precede the admission visit. The CPT code for such sick visit is a subsequent visit code. Some states and the Joint Commission for the Accreditation of Hospitals (JCAHO) have more stringent time rules for admission visits.

SUBSEQUENT VISIT CODES • History is divided into  History of present illness  Past medical history  Social history  Review of systems and for each of the four categories into  Problem-focused  Expanded problem focused  Detailed  Comprehensive • Examination is divided into  Problem focused  Expanded problem focused  Detailed  Comprehensive • Medical decision making is divided into  Number of diagnoses and management options  Amount and complexity of data  Risk of complication, morbidity, and mortality and for each of the following categories into  Straightforward  Low  Moderate  High The various permutations of the above categories define the CPT level. 99307 requires (2 of 3) a problem-focused history, a problem-focused exam, and straightforward or low-complexity decision making. 99308 requires (2 of 3) an expanded problem-focused history, an expanded problem-focused exam, and

moderate-complexity decision making. 99309 requires (2 of 3) a detailed history, a detailed exam, and moderate- or highcomplexity medical decision making. 99310 requires (2 of 3) a detailed history, a detailed exam, and high-complexity medical decision making. The task of determining the correct CPT code is often more complicated than managing the patient. The fact that Medicare allows for a visit to document only a history and examination without decision making (assessment and plan) for these codes makes no clinical sense. Therefore, rather than present the scores of possible permutations, clinical vignettes should be more useful for clinicians.

REGULATORY SUBSEQUENT VISITS • 99307: a stable patient with Alzheimer’s dementia treated with donepezil • 99308: a patient with dementia, hyperlipidemia, and hypertension who is doing well but whose medication must be adjusted to improve blood pressure control. • 99309: a patient with a past stroke, diabetes, hypertension, progressing renal failure, and osteoporosis who visited her nephrologist 2 weeks ago and requires a review of capillary glucose levels, the nephrology consultation, and changes in several chronic condition treatments.

SICK VISITS • 99307: diagnosis and treatment of pink eye or eczema. • 99308: evaluation and management of urinary tract infection, new pressure ulcer. • 99309: evaluation and management of chest pain, new delirium, a new stroke, pneumonia. • 99310 would generally be used for the evaluation and management of a life-threatening illness in the nursing home in lieu of the hospital. The existence of comorbidities will often result in more work and a higher CPT code. For example, a UTI in a diabetic patient has additional clinical implications (e.g., impact of UTI on glycemic control) and would justify the use of CPT code 99309 instead of 99308 so long as the documentation accounts for the added complexity of the situation.

NOTES • A change of physician does not allow for usage of a comprehensive CPT code even if the work is comprehensive. • Advance practice nurses (nurse practitioner) are not allowed to perform the admission work for skilled patients. They can admit nonskilled patients. They can alternate the subsequent regulatory visits with the attending physician and there is no limitation on the number of sick visits they can perform. Nurse practitioners will be paid by Medicare for the services they are allowed to perform. • All consultations must be ordered by the attending physician or practitioner who works with him or her. • Even professionals with ample experience in coding will sometimes disagree about what CPT code to use. The choice among CPT codes is often difficult because of the interplay of multiple chronic conditions. AUTHOR: TOM J. WACHTEL, M.D.

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Nursing Home, Attending Physician Role INTRODUCTION Providing medical care to nursing home patients differs from both the hospital setting and the outpatient setting. Hospitalized patients are acutely ill and are seen every day. Subspecialists in many fields are available for consultation on short notice when help is needed. Ambulatory patients are seen episodically for chronic disease management, health maintenance, or acute conditions, but they are generally independent and mobile and can usually carry out their physicians’ orders independently or with minimal formal or informal assistance. Three concepts highlight the nature of nursing home medical care: interdisciplinary, geriatric, and regulated. Nursing home patients are typically sicker than ambulatory patients (postacute care) or more debilitated (long-term care), but their usual condition does not require daily visits. However, this patient population’s usual condition requires the services of various professionals such as nurses, rehabilitation personnel, dietitians, social workers, personal care attendants, and others with whom the attending physician must interact by phone or face-to-face. This ongoing interaction is necessary both to receive information about the patient’s status and to order care. Nursing home physicians must be comfortable with interdisci-

plinary teamwork in order to perform effectively. In addition, nursing home physicians must be familiar with geriatric principles to practice competently in this environment. This requires familiarity with geriatric syndromes and with functional concepts (assessments and outcomes). Finally, nursing homes are highly regulated institutions. Many of the state and federal government regulations are the result of historical (and sometimes current) poor care. Attending physicians may not agree with every regulation (as we do not always agree with every speed limit), but failure to comply results in difficulties for the facility and its managing staff, including the medical director. By agreeing to accept responsibility for the medical care of a nursing home resident, the attending physician agrees implicitly or explicitly (Fig. A1-7) to comply with the various rules and regulations. The nuts and bolts of the nursing home attending physician are described in the model practice agreement displayed in Fig. A1-7. The most notable difference from practice in other settings is the need to work as a member of a care team whose functional captain is the nurse, although the attending physician writes the orders and the nurses and other professionals carry them out. Because physician presence in the

MODEL PRACTICE AGREEMENT The attending physician and the nursing facility share a common goal—the nursing facility expectation should be separated and signed off by the administrator or owner, the highest possible quality of life of the residents. Achieving the highest attainable quality of life for the resident requires the joint efforts of multiple disciplines, which must operate collaboratively. The attending physician provides medical care (primary function) and interacts with nursing, rehabilitation specialists, dietary, social services, and other professionals (collaborative function) in the context of a highly government-regulated environment (regulatory function). This complex structure requires physician knowledge, skills, attitudes, and activities that are not typically encountered in other practice settings. This must be understood and accepted. Attending physicians agree to perform the following: 1. Approve orders on the day of admission (may be done by phone) 2. Visit new patients within 48 hours of admission, unless the patient was examined by a physician within the 5 days prior to admission (e.g., a hospital transfer). In the latter case, with documentation, the attending physician has 7 days to visit the patient. 3. The admission visit is comprehensive. It should include a history, a physical examination, a review/assessment of all the diagnoses, a plan of care for each of the diagnoses, a general goal/purpose for the nursing facility stay, and other matters that may need addressing (e.g., advance directives). 4. The attending physician agrees to visit the resident no less than every 30 days for the first 3 months, and then no less than every 60 days (“regulatory visits”). The attending physician also agrees to visit the resident when a change in condition or an accident requires such a visit (“sick visits”). Alternate regulatory visits and any sick visits may be performed by a nurse practitioner (NP) or a physician assistant (PA). 5. The progress notes must be legible, indicate the purpose of the visit, document the findings (H&P or S&O), and contain an assessment (diagnoses) and plan (treatments). The notes must be signed and dated. 6. Over the course of a year, all the resident’s medical problems and treatments must be documented in progress notes. This can be done over the course of several visits, or the resident can have an annual “comprehensive visit.” 7. The attending physician must be available 24/7 personally or through a coverage arrangement that will be disclosed to the nursing facility. 8. The attending physician agrees to sign all regulatory forms or contact the facility’s medical director if he or she believes a form is incorrect or inappropriate. 9. The attending physician will participate in the discharge planning process of residents under care. 10. The attending physician will attend at least one annual meeting with the facility’s medical director. 11. The nursing facility agrees to provide high quality services to its residents. 12. The nursing facility agrees to inform the attending physician and the resident’s family of any change in condition in a timely manner. 13. The facility will inform the attending physician of patient-specific problems and systemic issues identified during usual care, quality improvement activities or state/federal inspections. 14. The nursing facility will make every effort to provide specialty consultants (e.g., psychiatry and podiatry) on site, and will arrange for transportation of residents for off-site consultations. 15. The attending physician will inform the medical director of any concerns regarding the care provided to residents by nursing home personnel. FIGURE A1-7 Model Practice Agreement

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Nursing Home, Attending Physician Role facility is intermittent, nursing home nurses have been described as the “eyes and ears” of the physician, but the physician must be aware of the abilities of individual nurses in obtaining accurate clinical information. The most important factor in gauging the quality of this information is prior experience in working with a particular nurse. Interaction with residents’ families (and friends) is equally important for transfer of information in both directions. Families need to know what to expect. Transitions are a time of particular patient vulnerability because the patient is new to the care team, and because the transfer of information is often incomplete between institutions (e.g., hospital and nursing home). Ideally, direct communication between nurses and between the physicians from both institutions should take place, but in practice many obstacles prevent such interactions. The prevalence of dementia is near 50% among long-term care patients who cannot provide reliable medical histories. The quality of the recorded medical informa-

tion is also variable for many reasons (multiple transitions, low priority, specialty care). Regulations determine the frequency of routine physician visits, but there are no limitations on the number of “sick” visits that attending physicians can make so long as such visits are “necessary” in the judgment of the physician. There are also no regulatory limitations on consultations, but few specialists visit nursing home patients who therefore must be transported to consultants’ offices. This requires nursing home attending physicians to be true generalists and often extend their scope of practice beyond what would be ordinary in their hospital or office practice.

SUGGESTED READING Dimant J: Roles and responsibilities of attending physicians in skilled nursing facilities, J Am Med Dir Assoc 4:231-243, 2003. AUTHOR: TOM J. WACHTEL, M.D.

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Nursing Home, Medical Director Role INTRODUCTION

SPECIFIC MEDICAL DIRECTOR JOB DESCRIPTION

A medical director is a physician who is responsible for overseeing certain aspects of medical care and services for an organization or a health care system. Hospitals have department chairs, chiefs of staff, division directors, or vice presidents for medical affairs. As the needs and the care of nursing home residents became more complex, the expertise of physicians was needed to complement the knowledge and skills of the nursing staff. Medicare through the OBRA ’87 regulations requires that all long-term care facilities designate a medical director. Interpretive guidelines describe some following duties: • Ensure that the facility provides appropriate care. • Ensure implementation of resident care policies. • Provide oversight of physician services. • Oversee overall clinical care and make sure it is adequate. • Correct inadequate medical care when such is identified or reported. • Consult with residents or their attending physicians when needed or requested.

QUALIFICATIONS: 1. Licensed physician. 2. Two years of experience in long-term care or specialized training in geriatrics or chronic disease/impaired populations. 3. Desirable attributes: a. Medical director certification or equivalent training in management. b. Be the attending physician for a caseload of patients in the facility that one directs, e.g., 10%. AREAS OF RESPONSIBILITY 1. General: a. Overall coordination, execution, and monitoring of physician/practitioner activities. b. Monitoring and evaluating the outcomes of the health care services (e.g., quality assurance [QA]). 2. Physician/practitioner oversight. a. Establish and implement a procedure to review physician/ practitioner credentials and grant privileges to be or remain on the list of attending. b. Establish rules that govern the performance of physicians/ practitioners. c. Make staff aware of those rules. d. Oversee physician/practitioner performance. Establish a formal procedure for such oversight (e.g., QA). 3. Define the scope of practice for physicians/practitioners (would usually use state/federal regulations). 4. Ensure physician performance in the following activities: a. Accept responsibility for the care of residents assigned to them. b. Perform timely admission procedures including review of medical records. c. Make periodic as well as other pertinent visits. d. Provide adequate ongoing medical coverage (i.e., 24/7). e. Provide appropriate medical care. f. Document care and do so legibly. g. Formulate and approve advance directives/end of life orders. h. Other (may be physician, resident, or facility specific). 5. Cover for the attending physician when the latter is unavailable or not performing appropriately. The medical director usually becomes the attending physician by default when no other physician on staff is available or willing to accept the patient. The medical director may become involved directly in the care of another physician’s patient under the following circumstances: a. Request by resident/family. b. Request by nursing home staff (e.g., a nurse). c. Request by another physician. d. When aware of a quality of medical care concern. e. When aware of a risk management issue. f. The patient or proxy must consent to involvement by another physician or the medical director as rapidly as possible. 6. Policies and procedures: the medical director is responsible for the content of those policies and procedures that fall under the physician’s domain. The medical director should “sign off” on those documents and monitor their implementation even though this is not required by law. The medical director should review policies and procedures that pertain to other types of health care professionals (e.g., nursing) but not be responsible for their implementation. Other policies and procedures should not be under medical director oversight. 7. Quality Improvement (QI): The medical director or her/his designee must attend the quality assurance meetings and be an active participant in the QI process. The medical director should review and sign the minutes produced during

GENERAL STATEMENT The role of the medical director can be broken down into three categories: • Domains of care that fall under physician expertise: physician/ practitioner services including timeliness of visits, appropriateness of orders, credentialing of physician/practitioners, availability of needed consultants, infection control, formulating advance directives, employee health issues, medical record maintenance (e.g., admission notes, progress notes, discharge summaries), treatment protocols, etc. The medical director should be actively involved in collaboration with the facility’s leadership (e.g., the administrator and the director of nursing) in the oversight of the above domains and shares responsibility with the leadership for satisfactory nursing home performance of those areas. If problems are discovered during inspections, quality assurance activities, or otherwise, the medical director should provide assistance and recommendations pertaining to corrective action plans. The medical director may need to intervene directly with staff physicians/practitioners who are not performing according to expectation. • Domains of care that are the primary responsibility of other health professionals but require some degree of medical director input (e.g., nursing, physical therapy, dietary, social work): the medical director should be aware of those departments’ policies and procedures and how they are fulfilling their function. If problems are identified internally (e.g., as a result of a mishap or during the quality assurance process) or by an external party (e.g., by state inspectors), the medical director should be informed and may be involved in helping the nursing home formulate plans to correct the problem(s), but should not be held responsible for the actual implementation of the corrective actions, given that the medical director has no authority over any nursing home employees and has no access to nursing home financial resources. • Domains of services to nursing home residents that should not be under medical director oversight or responsibility include laundry services, food services, plumbing, fire, and safety: physicians have no training and should not be expected to have any expertise in these areas. Therefore, if problems are identified in those areas (e.g., as a “deficiency” during an inspection), the medical director can be informed of those problems (as may be required by the regulatory process), but there should be no expectation from anyone that the medical director has a role in the plan or correction or that she or he can be held responsible for such correction.

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Nursing Home, Medical Director Role those meetings. The medical director should participate in the facility’s overall QI process including areas that are not in the medical domain because a physician is often the most knowledgeable member of the QA committee in the management and interpretation of statistical data among nursing home personnel. 8. Employee health oversight: nursing homes have varying policies concerning employee health. It is not expected that medical directors substitute for employees’ primary care physicians. What is expected is that the medical director establish or approve policies that cover employee immunization programs, that address diagnosis and treatment of infectious illnesses that could be transmitted to residents or other employees, that deal with conditions that could affect the performance of the worker, and that ensure that occupational safety measures are consistent with regulatory requirements. 9. Infection control: the medical director is expected to advise and consult with designated nursing staff regarding communicable diseases, infection control, and isolation procedures, and interact when appropriate with public health officials. 10. Review the reports that describe the findings of formal inspections by the state department of health. When deficiencies are identified, the medical director must be involved in the plan of correction of problems that are in the medical domain (e.g., F tag 501). The medical director should acknowledge corrective actions concerning problems that exist in other health care domains but not be expected to be accountable for their actual corrections. Medical directors should not be party to corrective measures that have nothing to do with the practice of medicine. 11. Other: a. Staff education (physician and other personnel). b. Participate in hiring/interview of key management personnel (e.g., administrator and director of nursing). c. Participate in important project development (e.g., a respirator unit). d. Interact as needed with state’s ombudsman. e. Interact as needed with resident families. MEDICAL DIRECTOR OVERSIGHT PLAN AND AUTHORITY The nursing home administrator and medical director will draft a plan that will describe how the medical director will carry out her/his responsibilities. This plan should be a written document that is part of the medical director’s employment contract. This contract should define the authority of the medical director. The medical director reports to the administrator and usually serves in an advisory capacity. A formal procedure should be created and updated by facility management staff and by the medical director to document performance. Periodic medical director performance evaluations should be carried out. MEDICAL DIRECTOR ACCOUNTABILITY Medical directors are held accountable to perform the job described in this chapter. Nursing homes are subjected to considerable oversight by government agencies and other parties (ombudsman, families of residents, etc.). The frail nature of nursing home residents may lead to events such as death, injuries, pressure ulcers, malnutrition, medication errors, etc., which could generate complaints about nursing homes, their medical directors, and their physicians. The state’s department of (public) health and the state’s board of licensure and discipline may be asked to adjudicate those complaints. Therefore, medical directors should maintain a written record of their activities, for example, in the form of a quarterly report to the QA committee.

CAVEATS The federal and state regulations define a broad outline of nursing home medical director responsibilities; however, those texts are so vague as to preclude a direct translation into a functional and realistic job description. Additionally, the breadth of the regulatory scope of responsibilities of the medical director job within the long-term care setting is unreasonable because it includes areas of involvement where physicians have no expertise. Finally, the authority bestowed on medical directors is limited by the part-time nature of the position and its advisory status without hire or firepower over any member of the nursing home employees. Pursuant to the Federal Nursing Home Reform Act of 1987 and specifically, 42 C.F.R. 483.75(I), each nursing home covered by the act must designate an individual to serve as a medical director. The regulations further state that each medical director is responsible for (1) the implementation of resident care policies and (2) the coordination of medical care in the facility. Although these subtopics may appear simple and straightforward, it is the variety of responsibilities included within each subtopic that cause concern and call for interpretation. Indeed, taken literally, the job description implied by the regulatory language goes beyond the role of a hospital chief of staff or department chair. For example, a nursing home medical director’s responsibility for the implementation of “resident care policies” has been interpreted to extend to “Admissions, transfers, and discharges; infection control; use of restraints; physician privileges and practice; responsibilities of non-physician health care workers in resident care, emergency care and resident assessment and care planning; accidents and incidents; ancillary services such as laboratory, radiology and pharmacy; use of medication; use and release of clinical information; and overall quality of care.” In addition, a director’s responsibility as related to “the coordination of medical care” in the facility has been interpreted to encompass “Monitoring and ensuring implementation of resident care policies, and providing oversight and supervision of physician services and medical care of residents; overseeing the overall clinical care of residents to ensure that care is adequate and effective; evaluating reports of possible inadequate medical care and taking appropriate action to remedy problems; and assuring the support of essential medical consultants as needed.” Despite the vast amount of responsibility imposed on each director, the regulations do not provide each medical director with an equivalent amount of authority necessary to ensure compliance with the pertinent regulations. Furthermore, most medical director contracts only require that the director work at the facility for a minimal number of hours (e.g., 2-4) each week. In combination with the regulations, such arrangements make the director an easy target for liability purposes, investigation by state licensing boards, and even criminal prosecution, but do not provide an obvious mechanism whereby the director can enforce his or her ideas and strategies. A carefully worded medical director employment contract may offer some protection (Fig. A1-8).

SUGGESTED READING Levenson S: The medical director: back to basics, Caring for the ages 4651, 2004. AUTHOR: TOM J. WACHTEL, M.D.

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Nursing Home, Medical Director Role SAMPLE MEDICAL DIRECTOR CONTRACT FOR RHODE ISLAND NURSING HOMES WHEREAS, Happy Care Health Center in order to participate in certain federal and/or state health care programs, is required to engage the services of a medical director and WHEREAS, Octoplusdoc, MD, hereinafter also referred to as the medical director, is a doctor of medicine licensed to practice his/her profession under the laws of the State of Rhode Island, is willing to provide said services as medical director, NOW, THEREFORE, said Happy Care Health Center and Dr. Octoplusdoc hereby and herewith mutually agree to the terms and conditions hereinafter set forth: 1. Happy Care Health Center designates Octoplusdoc, MD as medical director 2. The medical director shall devote XX hours per month (more or less) to the performance of his/her non-direct patient care duties under this agreement. 3. The medical director provides medical expertise and communicates regularly with the administrator, the director of nursing and other professional staff regarding clinical and administrative issues, specific patient care problems and professional staff needs. 4. The medical director will be involved and accountable in the development of medical treatment policies and procedures. The medical director should be aware of other health professionals’ policies (e.g., nursing, rehabilitation, dietician, social work). Nonclinical policies and procedures (e.g., cleaning, kitchen, plumbing, fire codes) are not the medical director’s responsibility. 5. The medical director participates in developing and is accountable for policies and procedures regarding the services of the medical and practitioner staff: 5.1 Credentialing of physicians and advance practice nurses/physician assistants 5.2 Define the scope of practice for physicians/practitioners 5.3 Establish rules that govern physician/practitioner performance: • Accept responsibility for the care of residents assigned to them • Perform timely admission visits, including examinations and writing orders • Perform periodic and other pertinent visits • Provide appropriate state of the art medical care • Document care consistent with CMS documentation guidelines • Formulate/approve end of life orders 5.4 In emergency situations, cover for the attending physician when unavailable or not performing appropriately 6. The medical director oversees the implementation of resident care policies and medical/practitioner staff performance 6.1 Participate in on-going quality improvement/assurance activities by attending the QI/QA committee meetings and reviewing the minutes 6.2 Advise on specific resident care problems identified during the normal process of delivering services to residents 6.3 Advise about the identification and management of systemic medical problems that may occur from time to time (e.g., outbreaks and infection control issues). 7. The medical director assists the facility in identifying needs for new resident care or staff policies/procedures and reviews existing policies from time to time. 8. The medical director advises the administrator and other relevant organization leaders about employee health policies and issues 9. The medical director must review state and federal survey/inspection reports and help to prepare a response and/or plan of correction when needed. 10. The medical director helps the facility to coordinate the care provided to residents. This becomes particularly important when a long-term care facility identifies itself as specialized and needs a relevant specialist to provide consultation (e.g., respirator unit, dialysis patients, etc.). The medical director cannot be held responsible for certain services that are unavailable (e.g,. dental care) 11. The medical director will work with the facility to promote residents’ rights and quality of life and interacts with ombudsman when needed. 12. The administrator and the director of nursing shall make themselves available to the medical director to discuss relevant matters as needed. 13. Facility personnel will inform the medical director about care issues in a timely manner as such issues arise. 14. The medical director provides a periodic report to the administrator and receives a periodic evaluation from the administrator. 15. This center shall pay to the medical director as a fee for his services, the amount of $XXXX per month. Said amount of money is compensation for his/her services as medical director only, and does not include any amounts due him/her for professional fees arising from his/her services to residents or others (e.g., employees) and payable to him/her by residents or third parties. In no case is the professional fee for individual care of a resident a part of the payment for services as medical director. 16. The medical director shall not be considered, and he/she is not, an employee of Happy Care Health Center, but is an independent contractor responsible for his/her own acts. 17. The medical director is expected to attend meetings, conferences, seminars or the like that may be concerned in whole or in part with medical direction in order to remain current with the job. The nursing home will cover up to $XXXX per year in expenses related to continuing education activities. 18. This agreement may be amended at any time by mutual agreement, in writing. This agreement shall be for a term of one year, but shall automatically renew on the anniversary date of its signing for an additional year each anniversary date thereafter. This agreement may be terminated by the medical director at any time with 60 days notice or by the nursing home at any time with 180 days notice (or continued payment for 180 days from the time of termination notice). 19. The medical director shall maintain malpractice insurance at all times in an amount not less than one million dollars ($1,000,000). This insurance will cover the medical director for his/her administrative duties (this is not typically included in standard medical malpractice policies). If the nursing home provides such coverage, the medical director will be identified by name in its policy. 20. Nothing in this contract shall be construed to prohibit either party from entering any other agreement with any other person or entity. FIGURE A1-8 Sample Medical Director Contract for Rhode Island Nursing Homes

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Occupational Therapy INTRODUCTION Occupational therapy (OT) is a health profession designed to help people improve their ability to perform tasks in their activities of daily living (ADL) and working environments. Occupational therapists work with individuals who have conditions that are mentally, physically, developmentally, or emotionally disabling. Occupational therapists help persons improve their basic motor functions and reasoning abilities as well as compensate for permanent loss of function. Their goal is to help clients have independent, productive, and satisfying lives. Occupational therapists assist patients in performing activities of all types, ranging from using a computer to caring for daily needs such as dressing, cooking, and eating. Physical exercises may be used to increase strength and dexterity, whereas other activities may be chosen to improve visual acuity and the ability to discern patterns. Therapists instruct those with permanent disabilities, such as spinal cord injuries, cerebral palsy, or muscular dystrophy, in the use of adaptive equipment, including wheelchairs, splints, and aids for eating and dressing. Family and caregiver education are core components of the field.

EDUCATION AND LICENSING • Currently a 4-year bachelor’s degree is the minimum requirement to be an occupational therapist registered (OTR). • Beginning in 2007, a master’s degree will become the minimum requirement to qualify for an OTR. • Occupational therapists must graduate from an accredited educational program and pass a national certification examination. • Licensing is at a state level. • Occupational therapy assistants have either a 2-year associate’s degree or a 1-year certificate program.

SCOPE OF OCCUPATIONAL THERAPY ASSESSMENT AND TREATMENT TECHNIQUES • Perceptual, sensory, and cognitive assessment. • Assessment of activities of daily living (ADL). • Assess work readiness.

• Assessment of need for assistive technology. • Driving assessments. • Design, fabrication, and training in the use of assistive technology and orthotic or prosthetic devices. • Customized treatment programs to improve a person’s ability to perform ADL. • Comprehensive home and job site evaluations with adaptation recommendations. • Cognitive retraining programs. • Guidance to family members and caregivers.

PHYSICIAN REFERRAL TO OCCUPATIONAL THERAPISTS • Referral can be for assessment, treatment, or both. • Referral needs a specific diagnosis. • Referral should include a statement requesting a specific treatment or that the therapist can evaluate and treat as he or she determines most appropriate.  Examples: 1. Dx. Stroke, ICD-9CM 436: evaluate ability to self-feed and provide large-diameter grip eating utensils, a plate guard, and two-handed cup. 2. Dx. Parkinson’s disease, ICD-9CM 332.0: evaluate and treat as necessary. • Occupational therapy is covered under Medicare and most health insurance plans with a physician’s prescription.

SUGGESTED READINGS American Medical Association: Health Professions Career and Education Directory, Chicago, 2002, Author, pp 248-249. Hoenig H, Cutson T: Geriatric rehabilitation. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 285-302. Mosqueda L, Brummel-Smith K: Rehabilitation. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 149-163. AUTHOR: JOHN B. MURPHY, M.D.

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Palliative Care INTRODUCTION In 1900 most Americans died of unexpected relatively acute medical illnesses, conditions, and accidents. Currently, most Americans die of complications of chronic illnesses. Palliative care as defined by the World Health Organization refers to “the active total care of patients whose disease is not responsive to curative treatments. Control of pain, of other symptoms, and psychological, social and spiritual problems is paramount. The goal of palliative care is achievement of the best possible quality of life for patients and their families.” Although palliative care is imperative and most commonly provided at the end of life, it should be recognized that for those living with chronic illnesses and disability, palliative care should be an essential component of care even though they may not be imminently dying. Currently fewer than 20% of Americans are enrolled in hospice before death.

PROCESS OF CARE • The physician needs to recognize that palliative care is appropriate. Such recognition should come from the physician’s assessment of the patient’s condition but may come to the physician’s attention from other health professionals, patients, family, or friends. • Following recognition that palliative care is appropriate, the physician needs to facilitate a discussion of palliative care and end of life decisions with the patient and family. This will frequently take more than one encounter and should include an assessment of what the patient and family understand, what their beliefs and wishes may be, and the provision of information regarding prognosis as well as a sense of confidence in the ability to provide relief from suffering. • The physician needs to establish a plan of care and help the family continue to adhere to the goals of the plan as circumstances change. With adequate support, patients and families can stick to the initial plan and avoid the emotional roller coaster of constantly changing goals. Establishing a plan of care should include involvement of those resources necessary to accomplish the goals (e.g., referral to a hospice program, involvement of clergy).

COMPONENTS OF PALLIATIVE CARE SYMPTOM CONTROL • Pain  Pain assessment—use pain assessment scale I—-I—-I—-I—-I—-I—-I—-I—-I—-I—-I 0 1 2 3 4 5 6 7 8 9 10 No Moderate Worst pain pain possible pain  Nonpharmacologic pain control—heat, cold, massage, cognitive behavioral interventions (relaxation, imagery, and psychotherapy)  Pharmacologic pain control 1. Mild pain—acetaminophen, NSAIDs (including aspirin). 2. Moderate pain—opioids: codeine, hydromorphone, morphine, hydrocodone, and oxycodone; administer extended release medications on schedule and shortacting medications for breakthrough pain. • Anxiety  Nonpharmacologic—cognitive-behavioral interventions (relaxation, imagery, and psychotherapy).  Pharmacologic—intermediate acting benzodiazepam (lorazepam), morphine.

• Constipation—stool softener (e.g., docusate sodium), bowel stimulant (e.g., senna), and if needed osmotic laxative (e.g., lactulose). • Nausea/Vomiting—treatment should be based on the site causing the nausea and vomiting:  Central chemoreceptor trigger center—use prochlorperazine or ondansetron.  Gut—use serotonin antagonists or antimotility agent.  Vestibular—use meclizine or scopolamine.  Cerebral cortex (raised intracranial pressure)—use dexamethasone. • Diarrhea: frequently secondary to anticonstipation medications or fecal impaction; if true diarrhea try cholestyramine. • Dyspnea—treat underlying cause, then oxygen and opioids. • Anorexia  Nonpharmacologic—oral agents like artificial saliva, glycerin swabs, and Popsicles.  Pharmacologic—megestrol acetate and corticosteroids may be helpful early in the illness. • Cough—treat underlying illness, then dextromethorphan and opioids. • Delirium—identify reversible causes (e.g., infection, impaction, pain, hypoxia) and then use antipsychotics. • Depression—SSRIs and possibly psychostimulants. CAREGIVER SUPPORT • Psychologic—assess for depression and anxiety and treat accordingly. • Spiritual—assess spiritual needs, incorporate spiritual needs into care plan, and involve appropriate consultants. • Social—assess for caregiver burnout, identify financial resources, consider respite care, and involve appropriate consultants (e.g., MSW).

SETTINGS • Home—most formal hospice care is provided in individual’s homes but this requires able family members or friends to provide the majority of custodial care. • Inpatient hospice—typically for terminal care, acute control of symptoms, or as bridge in home hospice program. • Nursing home—preferably provided with formal involvement of hospice program. • Hospital—common, but not optimum, site for terminal care.

FINANCES • Hospice—a benefit of Medicare that covers palliative care when elected by a patient and a physician certifies that the patient has a prognosis of 6 months or less (see Hospice in Appendix 1).  Covers physician services related to hospice diagnosis, nursing care, medications, durable medical equipment, social services, short-term respite care, chaplaincy services, physical and occupational therapy, bereavement services, and home health aid services.

SUGGESTED READINGS AGS Panel on Chronic Pain in Older Persons: The management of chronic pain in older persons, J Am Geriatr Soc 46(5):635-652, 1998. AGS Panel on Persistent Pain in Older Persons: The management of persistent pain in older persons, J Am Geriatr Soc 50:5205-5224, 2002. Hlorenz K, Lynn J: Care of the dying patient. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 323-334. AUTHOR: JOHN B. MURPHY, M.D.

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Physical Therapy INTRODUCTION Physical therapy is a health profession concerned with the assessment, diagnosis, and treatment of disease and disability through physical means. Physical therapy is performed by a licensed practitioner who works with the physical aspects of a medical illness and specializes in the use of exercise to treat physical conditions. Physical therapy focuses primarily on assessment and treatment of persons with musculoskeletal, neurologic, and cardiopulmonary conditions. The physical therapist may use a variety of modalities and prescribe devices. The goals are to improve strength, mobility, and function; relieve pain; and prevent or minimize disability. Patient and family education are core components of the practice of physical therapy.

EDUCATION AND LICENSING • Physical therapist has at minimum a 4-year college degree, often a master’s degree, and occasionally a doctorate. • Physical therapy assistant generally has a 2-year junior or community college associate’s degree. • Physical therapists are licensed at a state level but must pass a national exam administered at the state level. • Evaluate and treat primarily under the prescription of a patient’s physician.

SCOPE OF EVALUATION CAPABILITIES • • • • • • •

Joint range of motion Muscle strength and endurance Integrity of sensation and proprioception Muscle tone Functional ability Gait assessment Performance of activities of daily living (ADL)

PHYSICAL THERAPY TECHNIQUES • • • • •

Therapeutic exercise Joint mobilization and range of motion exercises Ambulation training Cardiovascular endurance training Relaxation exercises

• • • •

Therapeutic massage Biofeedback Training in ADL Pulmonary physical therapy

MODALITIES USED • • • • • •

Traction Ultrasound Cryotherapy Diathermy Electrotherapy Hydrotherapy

PHYSICIAN REFERRAL TO PHYSICAL THERAPISTS • Referral can be for assessment, treatment, or both. • Referral needs a specific diagnosis. • Referral should include a statement requesting a specific treatment or that the therapist can evaluate and treat as he or she determines most appropriate.  Examples: 1. Dx. Parkinson’s disease, ICD-9CM, 332.0: evaluate gait, prescribe and train patient in use of a wheeled walker. 2. Dx. Adhesive capsulitis, ICD-9CM, 726.0: evaluate and treat as necessary. • Physical therapy is covered under Medicare and most health insurance plans with a physician’s prescription, but primarily for recovery, not for maintenance programs.

SUGGESTED READINGS American Medical Association: Health Professions Career and Education Directory, Chicago, 2002, Author, pp 289-290. Hoenig H, Cutson T: Geriatric rehabilitation. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 285-302. Mosqueda L, Brummel-Smith K: Rehabilitation. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 149-163. AUTHOR: JOHN B. MURPHY, M.D.

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Quality of Life DEFINITION There is no universally accepted definition of quality of life. The World Health Organization defines quality of life as “an individual’s perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns. It is a broad ranging concept affected in a complex way by the person’s physical health, psychological state, personal beliefs, social relationships and their relationship to salient features of their environment.” Quality of life is a subjective determination, thus two individuals with the same physical status may have different quality of life perceptions.

COMPONENTS OF QUALITY OF LIFE Four domains have been found to affect the quality of life perceptions of older adults: 1. Physical: a composite of one’s bodily status (well versus ill), bodily and mental capabilities, and satisfaction of needs necessary to sustain life (e.g., food, shelter). 2. Psychologic: determined by the ability or inability to cope emotionally with one’s life situation. 3. Social: the presence of interpersonal relationships that form a network that is available to provide bodily and emotional support as needed. 4. Environmental: a composite of the location where an individual lives and the persons with whom the individual interacts in that location.

SYNONYMS Well-being. Health-related quality of life (HRQOL) is a specific term used to define the impact of health status on quality of life.

EPIDEMIOLOGY The factors found to be associated with quality of life vary from study to study. Some of the predictors associated with lower quality of life include hospitalization, poor health behaviors, impaired mobility, urinary incontinence, hemodialysis, and inability to complete basic or instrumental activities of daily living without assistance. Institutionalized older adults report lower quality of life compared with community-dwelling older adults. The association between gender or race and quality of life depends on the population examined. In two studies, women over the age of 75 reported lower quality of life than their male counterparts. African-American dialysis patients have been found to report higher quality of life compared with non—African-American dialysis patients. In contrast, a study conducted among terminally ill persons did not find any association between gender or race and quality of life perceptions.

PHYSICAL FINDINGS There are no physical findings that are specific to perceived quality of life. Nonspecific physical findings that have been associated to lower quality of life include body mass index 20 and poor dentition (20 teeth). A variety of symptoms including shortness of breath, chronic cough, anxiety, depression, fatigue, insomnia, and pain are also associated with lower quality of life.

EVALUATION OF QUALITY OF LIFE 1. Several generic and disease-specific instruments have been developed to assess quality of life. Generic instruments typically assess multiple domains and are applicable to individuals with a variety of illnesses. Disease-specific instruments assess domains that are presumed to relate directly to the target illness and are only applicable to individuals with the specific illness being targeted. Four quality of life instruments pertinent to older adults are described as follows. Please refer to Appendix 2 for more information.

a. Medical Outcomes Study Short Form 36-Item Questionnaire (SF-36) is a generic health status instrument that is often used in research studies to assess quality of life. The instrument is easy to administer and has been found to be suitable as an interviewer-administered questionnaire for older outpatients. Poor performance in studies that enrolled hospitalized older adults suggests the instrument should not be administered to this population. Adults aged 75 years and over may have difficulty selfadministering the SF-36. b. World Health Organization Quality of Life Instrument for Older Adults (WHOQOL-OLD) is a generic quality of life instrument that is currently under development by the World Health Organization. The WHOQOL-OLD is being designed as a research instrument for investigations that enroll older adults from a variety of cultures. The instrument will become available for general use in the near future. c. Missoula-VITAS Quality of Life Index (MVQOLI) is an instrument that was designed for use with hospice patients who are terminally ill because of any advanced disease process. As such, the questionnaire has an end of life focus. The instrument is suitable for older adults and individuals with a variety of educational backgrounds. The MVQOLI is applicable as either a clinical or a research tool and may be self-administered. d. Quality of Life in Alzheimer’s Disease Scale (QOL-AD) is an instrument that was designed to assess the quality of life of persons with Mini Mental State Examination scores between 10 and 25. The QOL-AD incorporates information obtained directly from the patient and supplemental information provided by a caregiver. This instrument is short (13 items) and may easily be used in clinical practice or as part of a research investigation. 2. Global quality of life perceptions are commonly assessed through the use of a simple, direct question such as, “How would you rate your overall quality of life? Would you say Excellent, Very Good, Good, Fair, or Poor?” There is fair correlation between this single global item and the longer quality of life scales.

TREATMENT Efforts to improve quality of life should be individualized and targeted toward the domains (physical, psychologic, social, environmental) that affect quality of life perceptions. Examples of domain-specific interventions that may improve quality of life are as follows: • The physical domain may be improved by the alleviation of symptoms and improved disease management. • The psychologic domain may be positively affected by pharmacologic interventions, such as antidepressant medications, and nonpharmacologic interventions, such as counseling. • Efforts to improve upon a patient’s social domain may require the recruitment of family or formal caregivers. • A referral to a supervised housing complex may improve the environmental domain.

DIFFERENTIAL DIAGNOSIS Poor quality of life can be associated with any acute or chronic illness; thus, a global quality of life assessment is warranted in any older adult. The approach to management of an older adult with complaints of poor quality of life involves several steps. An exploration of the patient’s perception of his or her physical, psychologic, social, and environmental domains may be assisted by the use of a generic or disease-specific instrument. The remainder of the evaluation should focus on the problematic domains identified by the patient. Complaints of a

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Quality of Life physical nature suggest a comprehensive geriatric evaluation is warranted to determine whether treatment modifications are in order. Particular conditions for assessment include failure to thrive, malnutrition, and cancer. Conditions to consider if deficits in the psychologic domain are revealed include depression, cognitive impairment, selfneglect, or physical abuse. Assistance from a geriatric case manager or social worker should be sought for individuals who present with deficits in their social or environmental domains.

REFERRAL Older adults with poor quality of life may benefit from a referral to a geriatrics specialist. Referral to a geriatric psychiatrist should be considered for individuals that present with predominantly emotional problems.

PEARLS & CONSIDERATIONS • Quality of life (QOL) is composed of four domains—physical, psychologic, social, and environmental. Health-related quality of life (HRQOL) is used to define the impact of an individual’s health status on quality of life perceptions. The terms are occasionally used interchangeably. • Quality of life is subjective. Thus quality of life determinations may not correspond to perceived or actual health status. • Quality of life perceptions are dynamic and heavily influenced by expectations. The closer your actual existence is to your expectations for your life, the better your quality of life is likely to be. • Quality of life is one component of quality end of life care. Quality end of life care is a major focus of palliative care. Refer to the chapter on palliative care for more detail on the interaction of quality of life with care at the end of life.

SUGGESTED READINGS Borglin G et al: Self-reported health complaints and their prediction of overall and health-related quality of life among elderly people, Int J Nursing Studies 42:147-158, 2005. Borowiak E, Kostka T: Predictors of quality of life in older people living at home and in institutions, Aging Clin Exp Res 16:212-220, 2004. Byock IR: Measuring quality of life for patients with terminal illness: the Missoula-VITAS quality of life index, Palliat Med 12:231-244, 1998. Detmar SB et al: Health-related quality of life assessment and patientphysician communication: a randomized controlled trial, JAMA 288:3027-3034, 2002. Farquhar M: Elderly people’s definitions of quality of life, Soc Sci Med 41:1439-1446, 1995. Ferrucci L et al: Disease severity and health-related quality of life across different chronic conditions, J Am Geriatr Soc 48:1490-1495, 2000. Hayes V et al: The SF-36 Health Survey Questionnaire: is it suitable for use with older adults? Age Ageing 24:120-125, 1995. Katsura H et al: Both generic and disease specific health-related quality of life are deteriorated in patients with underweight COPD, Resp Med 99:624-630, 2005. Lara-Munoz C, Feinstein AR: How should quality of life be measured? J Investig Med 47:17-24, 1999. Nanda U, Andresen EM: Health-related quality of life: a guide for the health professional, Eval Health Prof, 21:179-215, 1998. Parker SG et al: Measuring health status in older patients: the SF-36 in practice, Age Ageing 27:13-18, 1998. Stenzelius K et al: Patterns of health complaints among people 75 in relation to quality of life and need of help, Arch Geron Geriat 40:85102, 2005. Testa MA, Simonson DC: Assessment of quality-of-life outcomes, N Engl J Med, 334:835-840, 1996. WHOQOL Group: http://www.who.int/evidence/assessment-instruments/qol/ql1.htm AUTHOR: LISA M. WALKE, M.D.

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Rehabilitation INTRODUCTION • Rehabilitation is critical to geriatric health care because disabling conditions are common among older persons and greatly affect quality of life, but are frequently amenable to treatment. • Rehabilitation is based on a conceptual framework wherein a disease results in impairment, which is an alteration in physical function at the organ level. The impairment then causes a limitation in a person’s activities of daily living (ADL), which is known as a disability. When a disability prevents a person from fully functioning in society, the person has a handicap. This framework is depicted with stroke as an example. Disease: Stroke Impairment: Left side weakness (organ level) Disability: Inability to transfer (person level) Handicap: Inability to live alone (societal level) • Rehabilitation is an approach to care that can be applied anywhere and not just in special units. • Rehabilitation is less involved in treating disease and more involved in enhancing function across all domains; physical, psychological, and social. • Rehabilitation is based on comprehensive functional assessment and typically is interdisciplinary.

PROCESS FOR REHABILITATION • Stabilize the primary problem (e.g., stroke).  Treat the hypertension or atrial fibrillation to prevent another stroke. • Prevent secondary complications.  Prevent pressure ulcers, DVT, malnutrition, or a shoulder subluxation. • Restore lost function.  Strength and endurance training: teach stand pivot transfers from bed to wheelchair and instruct in the use of a walker or wheelchair. • Promote adaptation of the person to the environment.  Facilitate physical and psychological acceptance of the changes in function. • Adapt the environment, physical and personal.  Build a ramp for entry into the house, widen doorways to allow a wheelchair to enter, install grab bars in the bathroom.  Assess family strengths, build on strengths, and provide supports to bolster weaknesses (e.g., respite care, elderly day care).

REHABILITATION PROFESSIONALS • Physiatrist—physician trained in physical medicine and rehabilitation (PM&R). • Rehabilitation nurse—provides nursing care, reinforces and adapts techniques taught by other rehabilitation team members, provides psychologic support, and trains family and other caregivers. • Physical therapist—assesses strength, range of motion, endurance, and mobility. Focuses primarily on transfers and ambulation. • Occupational therapist—focuses on assessing and addressing disabilities in activities of daily living (ADL) and some instrumental activities of daily living (IADL) • Speech pathologist—evaluates and treats patients with aphasia, dysphagia, perceptual disorders, speech impairments, and cognitive problems. • Recreational therapist—identifies and implements satisfying recreational activities to help patients adjust to their disability. • Audiologist—assesses degree and characteristics of hearing loss, prescribes auditory aids (including hearing aids), and conducts aural rehabilitation.

• Psychologist—assesses psychologic function and treats psychologic conditions. • Neuropsychologist—assesses complex neuropsychologic impairments and develops plans for treatment, frequently assisting other rehabilitation team members in adapting their interventions to meet the unique needs of cognitively impaired patients. • Social worker—assesses social and psychologic strengths and weaknesses of patients and caregivers and identifies community resources needed for optimum family function. • Orthotist—fashions complex rehabilitation aids such as a prosthesis or back brace.

REHABILITATION SETTINGS • General principles  Provided in the least restrictive, most independent, and least costly setting  Provided only following a decrement of function, not for maintenance  Provided only as long as the patient continues to make progress  Provided only when a patient has sufficient cognitive capability to benefit from therapy • Acute hospital  Immediate phase post illness or operation  Duration—brief, a few days to a week  Focuses on initial assessment and determination of where to provide subsequent care  Designed to prevent complications and bridge patient to next setting  Examples—immediate period post stroke or hip fracture • Acute-level rehabilitation center  Patients with complex medical and/or rehabilitation needs that require an interdisciplinary team of rehabilitation specialists  Duration—intermediate, generally 1 to 3 weeks  Patient must require two or more therapies, be able to benefit from and participate in therapy for at least 3 hours per day  Unable to have needs met in a less intensive (expensive) setting  Example—stroke patient with good endurance, hemiparesis, mild cognitive deficits, and dysphagia • Skilled nursing facility (SNF)  Patients with functional losses that would benefit from rehabilitation but cannot be cared for at home because of the extent of the functional impairments or the lack of support in the home who may benefit from an interdisciplinary team  Duration—intermediate, weeks to a few months  Must need a licensed professional (registered nurse, physical or occupational therapist, or speech pathologist) 5 days per week  Example—patient with hip fracture who lives alone  Medicare requires a hospitalization of at least 3 days in duration within the previous 30 days • Home  Patients with functional losses that would benefit from rehabilitation who have adequate supports in the home or who have mild impairments  Duration—brief to long term, days to months  Rehabilitation does not require cumbersome equipment that cannot be provided in the home  Patient must be homebound (does not go out of the house except for medical care)  May require one or more therapies and may provide interdisciplinary team care

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Rehabilitation 

Example—patient who has a total hip replacement for osteoarthritis, is otherwise healthy and robust, who has excellent in-home support and a handicapped-accessible home • Outpatient  Straightforward rehabilitation needs, with one or more therapies, but commonly only one  Duration—brief to long term  May require access to sophisticated equipment  Frequently follows acute hospital, rehabilitation center, SNF, or home rehabilitation program  Example—gait assessment and prescription of adaptive mobility aid (e.g., cane or walker)

CONDITIONS NEEDING REHABILITATION AND LIKELY SETTING FOR PROVIDING SERVICES • Acute-level rehabilitation unit  Stroke, amputation, complex fracture (e.g., hip fracture in patient with previous stroke) • Skilled nursing facility  Routine hip fracture, total joint replacement, deconditioning

• Home  Stroke or major joint repair with excellent in-home supports, deconditioning • Outpatient  Follow-up care after rehabilitation in another setting, small or medium joint surgery, assessment of unstable gait, prescription of adaptive mobility aid (e.g., walker, cane), adhesive capsulitis of shoulder.

SUGGESTED READINGS Hoenig H: Geriatric rehabilitation: state of the art, J Am Geriatr Soc 45:1371, 1997. Hoenig H, Cutson T: Geriatric rehabilitation. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp. 285-302. Mosqueda L, Brummel-Smith K: Rehabilitation. In Ham R, Sloane P, Warshaw G (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp. 149-163. AUTHOR: JOHN B. MURPHY, M.D.

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Speech Pathology INTRODUCTION

PHYSICIAN REFERRAL TO SPEECH PATHOLOGISTS

Speech pathology is the science concerned with the diagnosis and treatment of functional and organic speech defects and disorders. Speech and language pathologists evaluate and treat patients with aphasia, cognitive problems, perceptual disorders, and dysphagia.

• • • •

EDUCATION AND LICENSING • Minimum education is master’s degree • National certification exam administered by the Educational Testing Service (ETS) • Licensing at the state level • Frequently will work with an audiologist

COMMON CONDITIONS ASSESSED AND TREATED • Dysphagia, including modified barium swallow examinations (MBS) • Aphasias • Stroke-associated cognitive and communication disorders • Language perception disorders • Hearing impairments and aural rehabilitation

Referral can be for assessment, treatment, or both. A referral needs specific diagnosis. The services must be medically necessary. The plan of care needs to be established by either a speechlanguage pathologist or the physician. • The patient must be under the care of a physician. • Medicare requires physician review of the plan of care every 30 days but no longer stipulates frequency of physician visits.

SUGGESTED READINGS Hoenig H, Cutson T: Geriatric rehabilitation. In Hazzard W et al: Principles of Geriatric Medicine and Gerontology, ed 5, New York, 2003, McGraw-Hill, pp 285-302. Mosqueda L, Brummel-Smith K: Rehabilitation. In Ham R et al (eds): Primary Care Geriatrics, ed 4, St. Louis, 2002, Mosby, pp 149-163. AUTHOR: JOHN B. MURPHY, M.D.

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Tools of Geriatric Assessment Alcohol Abuse Michigan Alcoholism Screening Test (MAST) Name: __________________________________________ Date:

__________________________________________

Score: __________________________________________ Yes

No

*1.

(2)

Do you feel you are a normal drinker? (By normal we mean you drink less than or as much as most other people)

❒

❒

2.

(2)

Have you ever awakened the morning after some drinking the night before and found that you could not remember part of the evening?

❒

❒

3.

(1)

Does your wife, husband, a parent, or other near relative ever worry or complain about your drinking?

❒

❒

*4.

(2)

Can you stop drinking without a struggle after one or two drinks?

❒

❒

5.

(1)

Do you ever feel guilty about your drinking?

❒

❒

*6.

(2)

Do friends or relatives think you are a normal drinker?

❒

❒

*7.

(2)

Are you able to stop drinking when you want to?

❒

❒

8.

(5)

Have you ever attended a meeting of Alcoholics Anonymous (AA)?

❒

❒

9.

(1)

Have you gotten into physical fights when drinking?

❒

❒

10.

(2)

Has your drinking ever created problems between you and your wife, husband, a parent, or other relative?

❒

❒

11.

(2)

Has your wife, husband, or other family member ever gone to anyone for help about your drinking?

❒

❒

12.

(2)

Have you ever lost friends because of your drinking?

❒

❒

13.

(2)

Have you ever gotten into trouble at work or school because of drinking?

❒

❒

14.

(2)

Have you ever lost a job because of drinking?

❒

❒

15.

(2)

Have you ever neglected your obligations, your family, or your work for two or more days in a row because you were drinking?

❒

❒

16.

(1)

Do you drink before noon fairly often?

❒

❒

17.

(2)

Have you ever been told you have liver trouble? Cirrhosis?

❒

❒

18.

(2)

After heavy drinking have you ever had Delirium Tremens (DTs) or severe shaking or heard voices or seen things that really weren’t there?**

❒

❒

Continued on following page

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Alcohol Abuse Michigan Alcoholism Screening Test (MAST) (Continued) 19.

(5)

Have you ever gone to anyone for help about your drinking?

❒

❒

20.

(5)

Have you ever been in a hospital because of drinking?

❒

❒

21.

(2)

Have you ever been a patient in a psychiatric hospital or on a psychiatric ward of a general hospital where drinking was part of the problem that resulted in hospitalization?

❒

❒

22.

(2)

Have you ever been seen at a psychiatric or mental health clinic or gone to any doctor, social worker, or clergyman for help with any emotional problem where drinking was part of the problem?

❒

❒

23.

(2)

***Have you ever been arrested for drunk driving, driving while intoxicated, or driving under the influence of alcoholic beverages? If yes, how many times? _______

❒

❒

24.

(2)

Have you ever been arrested, or taken into custody, even for a few hours, because of other drunk behavior? If yes, how many times? _______

❒

❒

*Negative responses are alcoholic responses. **5 points for each Delirium Tremens ***2 points for each arrest From Selzer ML: The Michigan Alcoholism Screening Test: the quest for a new diagnostic instrument, Am J Psychiatry 127:1653-1658, 1971.

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Cognition Blessed Dementia Scale and Information-Memory Concentration (IMC) Test Name: __________________________________________ Date:

__________________________________________

Address: ________________________________________ Phone: __________________________________________ D.O.B.: ___________________ Medical Record #:

Gender: Male

Female

________________________________

Changes in performance of everyday activities

1. Inability to perform household tasks 2. Inability to cope with small sums of money 3. Inability to remember short list of items, e.g., in shopping 4. Inability to find way about indoors 5. Inability to find way about familiar streets 6. Inability to interpret surroundings (e.g., to recognize whether in hospital or at home, to discriminate between patients, doctors and nurses, relatives and hospital staff, etc.) 7. Inability to recall recent events (e.g., recent outings, visits of relatives or friends to hospital, etc.) 8. Tendency to dwell in the past Changes in habits 9. Eating: Cleanly with proper utensils Messily with spoon only Simple solids, e.g., biscuits Has to be fed 10. Dressing: Unaided Occasionally misplaced buttons, etc. Wrong sequence, commonly forgetting items Unable to dress 11. Complete sphincter control Occasional wet beds Frequent wet beds Doubly incontinent Changes in personality, interests, drive No change 12. Increased rigidity 13. Increased egocentricity 14. Impairment of regard for feelings of others 15. Coarsening of affect 16. Impairment of emotional control, e.g., increased petulance and irritability 17. Hilarity in inappropriate situations 18. Diminished emotional responsiveness 19. Sexual misdemeanor (appearing de novo in old age) Interests retained 20. Hobbies relinquished 21. Diminished initiative or growing apathy 22. Purposeless hyperactivity

Information–Memory–Concentration Test 1 1 1

1⁄

2

1⁄

2

1⁄

2

1 1 1

1⁄

2

1⁄

2

1⁄

2

0 0 0

1

1⁄

2

0

1

1⁄

2

0

0 0 0

0 2 2 3 0 1 2 3 0 1 2 3 0 1 1 1 1 1 1 1 1 0 1 1 1

Total _______________ Ascertain from relative/friend. Applies to last 6 months. Score lies between 0 (fully preserved capacity) and +28 (extreme incapacity)

Information test—Score for correct response shown; incorrect responses score 0. Name 1 Age 1 Time (hour) 1 Time of day 1 Day of week 1 Date 1 Month 1 Season 1 Year 1 Place— Name 1 Street 1 Town 1 Type of place (e.g,. home, hospital, etc.) 1 Recognition of persons (cleaner, doctor, 2 nurse, patient, relative; any two available) Total _______________ Memory: (1) personal Date of birth Place of birth School attended Occupation Name of sibs or name of wife Name of any town where patient had worked Name of employers

1 1 1 1 1 1 1

(2) non-personal Date of World War I1 Date of World War II1 Monarch2 Prime Minister3

1 1 1 1

(3) Name and address (5-minute recall) Mr. John Brown 42 West Street Gateshead4

5

Concentration Months of year backwards Counting 1-20 Counting 20-1

2 2 2

1 1 1

0 0 0

Total _______________ Scores lie between 0 (complete failure) and +37 (full marks) 1. 1⁄2 for approximation within 3 years. 2. President in U.S. version. 3. Vice President in U.S. version. 4. Chicago in U.S. version.

From Blessed G, Tomlinson BF, Roth M: The association between quantitative measures of dementia and of senile change in the cerebral grey matter of elderly subjects, Br J Psychiatry 114:797-811, 1968.

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Cognition Clock Drawing Test (CLOX) An executive clock drawing task© STEP 1: Turn this form over on a light-colored surface so that the circle below is visible. Have the subject draw a clock on the back. Instruct him or her to “Draw me a clock that says 1:45. Set the hands and numbers on the face so that a child could read them.” Repeat the instructions until they are clearly understood. Once the subject begins to draw, no further assistance is allowed. Rate this clock (CLOX 1). STEP 2: Return to this side and let the subject observe you draw a clock in the circle below. Place 12, 6, 3, and 9 first. Set the hands again to 1:45. Make the hands into arrows. Invite the subject to copy your clock in the lower right-hand corner. Score this clock (CLOX 2). RATING Organization Elements

Point Value

Does figure resemble a clock?

1

Outer circle present?

1

Diameter  1 inch?

1

All numbers inside the circle?

1

12, 6, 3, and 9 placed first?

1

Spacing intact? (Symmetry on either side of the 12-6 axis?) If yes, skip next.

2

If spacing errors are present, are there signs of correction or erasure?

1

Only Arabic numerals?

1

Only numbers 1–12 among the Arabic numerals present?

1

Sequence 1–12 intact? No omissions or intrusions.

1

Only two hands present?

1

All hands represented as arrows?

1

Hour hand between 1 and 2 o’clock?

1

Minute hand longer than hour?

1

None of the following:

1

CLOX 1

CLOX 2

1) Hand pointing to 4 or 5 o’clock? 2) “1:45” present? 3) Intrusions from “hand” or “face” present? 4) Any letters, words, or pictures? 5) Any intrusion from circle below? TOTAL CLOX scores range from 0–15. Lower scores reflect greater impairment.

From Royall DR, Cordes JA, Polk M: CLOX: an executive clock drawing task, J Neurol Neurosurg Psychiatry 64:588–594, 1998. (http://jnnp.bmjjournals.com/cgi/reprint/64/5/588)

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Cognition Hachinski Ischemic Score (HIS) for Multi-Infarct Dementia Name: __________________________________________ Date:

__________________________________________

Address: ________________________________________ Phone: __________________________________________ D.O.B.: ___________________ Medical Record #:

Gender: Male

Female

________________________________

Abrupt onset Stepwise deterioration Fluctuating course Nocturnal confusion Relative preservation of personality Depression Somatic complaints Emotional incontinence History of hypertension History of strokes Generalized atherosclerosis Focal neurological symptoms Focal neurological signs (A score of greater than 7 suggests a vascular component to the dementia.) From Hachinski VC, Iliff LD, Silhka E, et al: Cerebral blood flow in dementia, Arch Neurol 32:632–637, 1975.

Score 2 1 2 1 1 1 1 1 1 2 1 2 2

509

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510

Cognition Mini-Cog Scoring Algorithm Mini-Cog

3-Item Recall  12

3-Item Recall  0

3-Item Recall  3 NONDEMENTED

DEMENTED CDT Abnormal DEMENTED

CDT Normal NONDEMENTED

CDT, Clock drawing test. From Borson S, Scanlon J, Brush M, Vitaliano P, Dokmak A: The mini-cog: a cognitive “vital signs” measure for dementia screening in multilingual elderly, Int J Geriatr Psychiatry 15:1021–1027, 2000.

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Cognition Mini-Mental State Examination (MMSE) The Mini-Mental State Examination (MMSE) is no longer available for text publication. The MMSE is now published and sold by Psychological Assessment Resources, Inc., 16204 N. Florida Ave, Lutz, FL 33549; phone: 1-800-331-8378 or 1-813-968-3003; website: http://www.minimental.com/. The standard MMSE form published by Psychological Assessment Resources, Inc. is based on its original 1975 conceptualization, with minor subsequent modifications by the authors. MMSE kits are available and include an MMSE clinical guide, pocket norms card, user’s guide, and test forms. The MMSE is also freely available at numerous online sites, including: • http://www.medafile.com/mmses.htm • http://www.medal.org Targeted Population: Older adults. Description: One of the most widely used tests of cognitive function, the MMSE is also well studied. The MMSE is a screening test only; diagnosis of dementia requires a full mental status examination with history and physical examination. The test contains 11 items in two parts. The first part requires verbal responses and assesses orientation, memory, and attention. The second part evaluates naming of objects, ability to follow oral and written commands, sentence writing, and ability to copy a complex polygon. Scores: Points are assigned to correct responses. Total scores range from 0 to 30. The mean score for normal individuals is 27.6; for patients with dementia, the mean score is 9.7 (Folstein et al, 1975). The cutoff score ranges from 23 to 25 in multiple studies (McDowell & Newell, 1996). Cutoff scores may vary based on level of education. Scores at or less than the cutoff warrant further evaluation of cognitive function. Accuracy: The MMSE has demonstrated good to excellent reliability (internal consistency, test–retest, and interrater) and validity (content and correlations with other cognitive function tests) in numerous studies (Tombaugh & McIntyre, 1992; McDowell & Newell, 1996). Predictive validity studies have shown that the MMSE has good ability to indicate when an individual becomes cognitively impaired. Some variation in scores was noted when educational levels were compared, with lower scores implying more cognitive impairment among those with higher education than among those with less education (Uhlmann & Larson, 1991). As education level increases, the specificity of the MMSE also rises, whereas the sensitivity decreases. Hence, cutoff scores may be lower in less educated persons. With the use of normative data, Crum et al (1993) provided cutoff scores of 19 for 0 to 4 years of education, 23 for 5 to 8 years of education, 27 for 9 to 12 years of education, and 29 for college level and beyond. Administration Time: This test is not timed but takes approximately 10 minutes to administer. Descriptive summary from Loretz L: Primary Care Tools for Clinicians: A Compendium of Forms, Questionnaires, and Rating Scales for Everyday Practice, St. Louis, 2005, Mosby.

511

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Cognition Modified Mini-Mental State Examination (3MS)

From Teng EL, Chui HC: The Modified Mini-Mental State (3MS) Examination, J Clin Psychiatry 48:8, 1987.

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Cognition Severe Mini-Mental State Examination

513

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Cognition

From Harrell LE, Marson D, Chatterjee A, Parrish JA: The severe mini-mental state examination: a new neuropsychologic instrument for the bedside assessment of severely impaired patients with Alzheimer disease, Alzheimer Dis Assoc Disord 14(3):168–175, 2000.

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Delirium Confusion Assessment Method (CAM)

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Delirium

From Inouye AK, van Dyck Ch, Alessi CA: Clarifying confusion: the confusion assessment method, Ann Intern Med 113(12):941–948, 1990.

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Delirium Confusion Assessment Method for the Intensive Care Unit (CAM-ICU) Confusion Assessment Method for the Intensive Care Unit (CAM-ICU) Instrument Delirium is diagnosed when both features 1 and 2 are positive, along with either feature 3 or 4. Feature 1: Acute Onset of Mental Status Changes or Fluctuating Course  Is there evidence of an acute change in mental status from the baseline?  Did the (abnormal) behavior fluctuate during the past 24 hours, that is, tend to come and go or increase and decrease in severity? Sources of information: Serial Glasgow Coma Scale or sedation score ratings over 24 hours as well as readily available input from the patient’s bedside critical care nurse or family. Feature 2: Inattention  Did the patient have difficulty focusing attention?  Is there a reduced ability to maintain and shift attention? Sources of information: Attention screening examinations by using either picture recognition or Vigilance A random letter test (see Attention Screening Examinations [ASE] for the Intensive Care Unit). Neither of these tests requires verbal response, and thus they are ideally suited for mechanically ventilated patients. Feature 3: Disorganized Thinking  Was the patient’s thinking disorganized or incoherent, such as rambling or irrelevant conversation, unclear or illogical flow of ideas, or unpredictable switching from subject to subject?  Was the patient able to follow questions and commands throughout the assessment? 1. “Are you having any unclear thinking?” 2. “Hold up this many fingers.” (Examiner holds two fingers in front of the patient.) 3. “Now, do the same thing with the other hand” (not repeating the number of fingers). Feature 4: Altered Level of Consciousness  Any level of consciousness other than “alert.”  Alert—normal, spontaneously fully aware of environment and interacts appropriately  Vigilant—hyperalert  Lethargic—drowsy but easily aroused, unaware of some elements in the environment, or not spontaneously interacting appropriately with the interviewer; becomes fully aware and appropriately interactive when prodded minimally  Stupor—difficult to arouse, unaware of some or all elements in the environment, or not spontaneously interacting with the interviewer; becomes incompletely aware and inappropriately interactive when prodded strongly  Coma—unarousable, unaware of all elements in the environment, with no spontaneous interaction or awareness of the interviewer, so that the interview is difficult or impossible even with maximal prodding Attention Screening Examinations (ASE) for the Intensive Care Unit (A) Picture Recognition ASE (B) Vigilance A Random Letter Test (A) Picture Recognition ASE (Fig. A2-1) Step 1: Five pictures Say to the patient: “Mr. or Mrs. X, I am going to show you pictures of some common objects. Watch carefully and try to remember each picture because I will ask what pictures you have seen.” Then show Step 1 of either Form A or Form B, alternating daily if repeat measures are taken. Step 2: Ten pictures Say to the patient: “Now I am going to show you some more pictures. Some of these you have already seen and some are new. Let me know whether or not you saw the picture before by nodding your head yes (demonstrate) or no (demonstrate).” Then show Step 2 of Form A or B, depending on which form was used in Step 1. Form A (Step 1) key cup car table hammer

Form B (Step 1) boot dog knife pants paintbrush

Form A (Step 2) key cup car table hammer glass lock truck chair saw

Form B (Step 2) boot dog knife pants paintbrush fork cat dress toothbrush shoe

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Delirium This test is scored by the number of correct “yes” or “no” answers (out of a possible 10) during the second step. To improve the visibility for elderly patients, the images are printed on 6  10-inch buff-colored paper and laminated with a flat finish. As did Hart et al, we showed each image for 3 seconds. When a patient had known visual impairment and no corrective lenses, we substituted the Vigilance A Random Letter Test (1, 2). (B) Vigilance A Random Letter Test Directions: Tell the patient: “I am going to read you a long series of letters. Whenever you hear the letter A, indicate by squeezing my hand.” Read the following letter list in a normal tone at a rate of one letter per second. LTPEAOAICTDALAA ANIABFSAMRZEOAD PAKLAUCJTOEABAA ZYFMUSAHEVAARAT Scoring: Currently, only preliminary standardized norms exist for this test. The average person should complete the task without error (x  0.2); a sample of randomly selected brain-damaged patients made an average of ten errors. Examples of common organic errors are a) failure to indicate when the target letter has been presented (omission error); b) indication made when a nontarget letter has been presented (commission error); and c) failure to stop tapping with the presentation of subsequent nontarget letters (perseveration error) (3)

REFERENCES 1. Hart RP, Levenson JL, Sessler CN, Best AM, Schwartz SM, Rutherford LE: Validation of a cognitive test for delirium in medical ICU patients, Psychosomatics 37(6):533-546, 1996. 2. Hart RP, Best AM, Sessler CN, Levenson JL: Abbreviated cognitive test for delirium, J Psychosom Res 43(4):417-423, 1997. 3. Strub RL, Black FW: The Mental Status Examination in Neurology, ed 3, Philadelphia, 1993, FA Davis.

FIGURE A2-1 Form A and Form B, Picture Recognition Attention Screening Examination for the Intensive Care Unit.

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Delirium Criteria Used by Delirium Experts Criteria for delirium from Diagnostic and Statistical Manual for Mental Disorders—Reference standard evaluations were performed using all available information including patient examinations and interactions, nurse and family interviews, physicians’ and nurses’ notes, laboratory values, and any other chart data present. A. Disturbance of consciousness (i.e., reduced clarity of awareness of the environment) with reduced ability to focus, sustain, or shift attention B. A change in cognition (e.g., memory deficit, disorientation, language disturbance) or the development of a perceptual disturbance that is not better accounted for by a preexisting, established, or evolving dementia C. Disturbance that develops over a short period of time (usually hours to days) and tends to fluctuate during the course of the day D. Evidence from the history, physical examination, or laboratory findings that the disturbance is caused by one of the following: i. Direct physiologic consequences of a general medical condition ii. Direct result of medication use or substance intoxication (substance intoxication delirium) iii. Direct result of a withdrawal syndrome (substance withdrawal delirium) iv. Direct result of more than one of the preceding etiologies (delirium due to multiple etiologies) The diagnosis of cognitive impairment involved careful observations of the abilities of the patient and knowledge of the patient’s former level of functioning. To identify all cases of cognitive impairment, we adopted the following measures: a) The preceding criteria from the Diagnostic and Statistical Manual for Mental Disorders and mental status definitions were employed consistently. b) A delirium expert evaluation was conducted to determine which of these criteria were met by the patient. This involved a bedside evaluation and screening for cognitive and attention deficits. c) Last, interviewing the family and nurse who provided the majority of patient care established baseline functioning and identified fluctuations.1

REFERENCE 1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, ed 4, Washington, DC, 1994, American Psychiatric Association. From Ely EW, Margolin R, Francis J, et al: Evaluation of delirium in critically ill patients: validation of the Confusion Assessment Method for the Intensive Care Unit (CAM-ICU), Crit Care Med 29(7):1370–1379, 2001.

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Depression and Anxiety Center for Epidemiologic Studies Depression Scale (CES-D) Name: __________________________________________ Date:

__________________________________________

As I read the following statements, please tell me how often you felt or behaved this way. IN THE LAST WEEK. Did you feel this way: 0  Rarely or none of the time (i.e., less than 1 day)? 1  Some or a little of the time (i.e., 1-2 days)? 2  Occasionally or a moderate amount of time (i.e., 3-4 days)? 3  Most or all of the time (i.e., 5-7 days)? —  No response

Rarely

Some of of the time

1. I was bothered by things that usually don’t bother me

0

1

2

3

—

2. I did not feel like eating; my appetite was poor

0

1

2

3

—

3. I felt that I could not shake off the blues even with help from my family and friends

0

1

2

3

—

3

2

1

0

—

5. I had trouble keeping my mind on what I was doing

0

1

2

3

—

6. I felt depressed

0

1

2

3

—

*4. I felt that I was just as good as other people

7. I felt that everything I did was an effort

Most of Occasionally the time

No response

0

1

2

3

—

3

2

1

0

—

0

1

2

3

—

10. I felt fearful

0

1

2

3

—

11. My sleep was restless

0

1

2

3

—

*12. I was happy

3

2

1

0

—

13. I talked less than usual

0

1

2

3

—

*8. I felt hopeful about the future 9. I thought my life had been a failure

14. I felt lonely

0

1

2

3

—

15. People were unfriendly

0

1

2

3

—

*16. I enjoyed life

3

2

1

0

—

17. I had crying spells

0

1

2

3

—

18. I felt sad

0

1

2

3

—

19. I felt people disliked me

0

1

2

3

—

20. I could not get going

0

1

2

3

—

*Items are reverse-scored. Range of scores 0–60, with higher scores indicating more depressive symptoms. From Radloff LS: The CES-D Scale: a self-report depression scale for research in the general population, Appl Psychol Measure 2:385–401, 1977.

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Depression and Anxiety Cornell Scale for Depression in Dementia (CSDD) Name: __________________________________________ Date:

__________________________________________

Scoring (based on symptoms/signs occurring during the week prior to testing): a  unable to evaluate; 0  absent; 1  mild or intermittent; 2  severe. Location: ❒ Nursing Home Resident 1. 2. 3. 4.

5. 6. 7. 8.

9. 10. 11.

12. 13. 14. 15.

16. 17. 18. 19.

❒

Outpatient

❒

Inpatient

A. MOOD-RELATED SIGNS ANXIETY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a anxious expression, ruminations, worrying SADNESS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a sad expression, sad voice, tearfulness LACK OF REACTIVITY TO PLEASANT EVENTS . . . . . . . . . . . . . . . . . . . . . . . . . .a IRRITABILITY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a easily annoyed, short tempered B. BEHAVIORAL DISTURBANCES AGITATION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a restlessness, handwringing, hairpulling RETARDATION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a slow movements, slow speech, slow reactions MULTIPLE PHYSICAL COMPLAINTS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a (score 0 if gastrointestinal symptoms only) LOSS OF INTEREST . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a less involved in usual activities (score only if change occurred acutely—in less than 1 month) C. PHYSICAL SIGNS APPETITE LOSS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a eating less than usual WEIGHT LOSS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a (score 2 if greater than 5 lb in one month) LACK OF ENERGY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a fatigues easily, unable to sustain activities (score only if change occurred acutely—in less than 1 month) D. CYCLIC FUNCTIONS DIURNAL VARIATION ON MOOD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a symptoms worse in the morning DIFFICULTY FALLING ASLEEP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a later than usual for this person MULTIPLE AWAKENINGS DURING SLEEP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a EARLY MORNING AWAKENING . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a earlier than usual for this person E. IDEATIONAL DISTURBANCES SUICIDE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a feels life is not worth living, has suicidal wishes, or makes suicidal attempt POOR SELF-ESTEEM . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a self-blame, self-deprecation, feelings of failure PESSIMISM . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a anticipation of the worst MOOD-CONGRUENT DELUSIONS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .a delusions of poverty, illness, or loss

Score: _____________________ Total score  8 suggests significant depressive symptoms.

0

1

2

0

1

2

0 0

1 1

2 2

0

1

2

0

1

2

0

1

2

0

1

2

0

1

2

0

1

2

0

1

2

0

1

2

0

1

2

0 0

1 1

2 2

0

1

2

0

1

2

0

1

2

0

1

2

521

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522

Depression and Anxiety The Cornell Scale is administered in two steps. First, the clinician interviews the patient’s caregiver on each of the 19 items of the Cornell Scale and then briefly interviews the patient. Symptoms and signs are described to the caregiver as they appear on the scale. For example, to rate the presence of anxiety, the clinician asks the caregiver whether or not he or she has observed the patient having an anxious expression, or whether or not the patient is ruminating or worrying. The clinician is free to use additional descriptions to help the caregiver understand the meaning of each item. The caregiver is instructed to base his or her report on observations of the patient’s behavior during the week prior to interview. Two of the items, “loss of interest” and “lack of energy,” require not only that the patient is less involved in usual activities or has less energy during the week prior to interview, but also that changes in these behaviors occurred relatively acutely, i.e., over a period of less than 1 month. In these two items, the caregiver is initially asked to describe the patient’s behavior during the week prior to interview and then is asked to provide information about the onset of behavioral changes that may have occurred earlier. The item on “weight loss” is based entirely on information about the patient’s weight during the month preceding the interview. During the interview with the caregiver, the clinician assigns preliminary scores to each item of the Corrnell Scale. Next, the clinician briefly interviews the patient using Cornell Scale items as a basis for inquiry and observation, but does not necessarily confine himself or herself to these questions. If there is a large discrepancy between the clinician’s observations and the caregiver’s report on any item, the clinician interviews the caregiver again and attempts to clarify the reason for disagreement. After this process, the Cornell Scale is scored on the basis of the clinician’s final judgment. The total time of administration and rating of the Cornell Scale is approximately 30 min (approximately 20 min interview with the subject’s caregiver and about 10 min interview with the subject). Reprinted with permission from Alexopoulos GS, Agrams RC, Young RC, et al: Cornell Scale for depression dementia, Biol Psychiatry 23(3):271–284, 1988. Copyright 1988 The Society of Biological Psychiatry.

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Depression and Anxiety Geriatric Depression Scale (GDS) Name: __________________________________________ Date:

__________________________________________

Circle the best answer for how you felt over the past week. 1. *Are you basically satisfied with your life?. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

2. Have you dropped many of your activities and interests? . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

3. Do you feel that your life is empty? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

4. Do you often get bored? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

5. *Are you hopeful about the future? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

6. Are you bothered by thoughts you can’t get out of your head? . . . . . . . . . . . . . . . . . . . . . . Yes

No

7. *Are you in good spirits most of the time?. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

8. Are you afraid something bad is going to happen to you? . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

9. *Do you feel happy most of the time? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

10. Do you often feel helpless?. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

11. Do you often get restless and fidgety? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

12. Do you prefer to stay at home, rather than going out and doing new things?. . . . . . . . . . . Yes

No

13. Do you frequently worry about the future? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

14. Do you feel you have more problems with memory than most? . . . . . . . . . . . . . . . . . . . . . Yes

No

15. *Do you think it is wonderful to be alive now? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

16. Do you often feel downhearted and blue? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

17. Do you feel pretty worthless the way you are now? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

18. Do you worry a lot about the past? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

19. *Do you find life very exciting? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

20. Is it hard for you to get started on new projects? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

21. *Do you feel full of energy? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

22. Do you feel that your situation is hopeless? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

23. Do you think that most people are better off than you are? . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

24. Do you frequently get upset over little things? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

25. Do you frequently feel like crying? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

26. Do you have trouble concentrating? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

27. *Do you enjoy getting up in the morning? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

28. Do you prefer to avoid social gatherings? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

29. *Is it easy for you to make decisions? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

30. *Is your mind as clear as it used to be? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yes

No

Scoring: Assign one point for each ‘yes’ answer: 2, 3, 4, 6, 8, 10, 11, 12, 13, 14, 16, 17, 18, 20, 22, 23, 24, 25, 26, 28 *Assign one point for each ‘no’ answer: 1, 5, 7, 9, 15, 19, 21, 27, 29, 30 Score: ______________

Completed by: ______________________________________

Date: _______________

Normal: 0 to 10; Mildly depressed: 11 to 20; Very depressed: 21 to 30 Adapted from Yesavage JA, Brink TL: Development and a validation of a geriatric depression screening scale: a preliminary report, J Psychiatr Res 17(1):37–49, 1983.

523

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Depression and Anxiety Hamilton Anxiety Rating Scale (HARS) Name: __________________________________________ Rater: __________________________________________ Date:

__________________________________________

The Hamilton Anxiety Scale (HARS, HAM-A) consists of 14 items, each defined by a series of symptoms. Each item is rated on a 5-point scale: 0-absent; 1-mild; 2-moderate; 3-severe; 4-incapacitating. Higher scores indicate increased anxiety. SYMPTOMS

1. Anxious Mood

0 1 2 3 4

• worries • anticipates worst

2. Tension • • • •

• • • •

• • • •

10. Respiratory Symptoms

0 1 2 3 4 11. Gastrointestinal Symptoms • • • •

12. Genitourinary Symptoms

0 1 2 3 4 13. Autonomic Symptoms

0 1 2 3 4

• decreased interest in activities • anhedonia • insomnia

7. Somatic Complaints–Muscular

0 1 2 3 4

• urinary frequency or urgency • dysmenorrhea • impotence

• poor concentration • memory impairment

6. Depressed Mood

0 1 2 3 4

dysphagia nausea or vomiting constipation weight loss

0 1 2 3 4

• difficulty falling asleep or staying asleep • difficulty with nightmares

5. Intellectual

0 1 2 3 4

• chest pressure • choking sensation • shortness of breath

fear of the dark fear of strangers fear of being alone fear of animal

4. Insomnia

0 1 2 3 4

tachycardia palpitations chest pain sensation of feeling faint

0 1 2 3 4

startles cries easily restless trembling

3. Fears

9. Cardiovascular Symptoms

• • • •

14. Behavior at Interview

0 1 2 3 4

0 1 2 3 4

dry mouth flushing pallor sweating

0 1 2 3 4

• fidgets • tremor • paces

• muscle aches or pains • bruxism

8. Somatic Complaints–Sensory • tinnitus • blurred vision

0 1 2 3 4 TOTAL SCORE: ______________

Adapted from Hamilton M: The assessment of anxiety states by rating, Brit J Med Psychol 32:50–55, 1959. Copyright 1959 The British Psychological Society. Reprinted with permission.

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Falls Abnormal Involuntary Movement Scale (AIMS)— Screening for Tardive Dyskinesia Patient Name: ____________________________________ Date of Visit:

Examiner: ___________________________________________

____________________________________

Examination Procedurea

Either before or after completing the EXAMINATION PROCEDURE observe the patient unobtrusively, at rest (e.g., in the waiting room). The chair to be used in this examination should be a hard, firm one without arms.

1) Ask patient whether there is anything in his/her mouth (e.g., gum, candy, etc.) and if there is, to remove it. 2) Ask patient about the current condition of his/her teeth. Ask patient if he/she wears dentures. Do teeth or dentures bother patient now? 3) Ask patient whether he/she notices any movements in mouth, face, hands, or feet. If yes, ask to describe and to what extent they currently bother patient or interfere with his/her activities. 4) Have patient sit in chair with hands on knees, legs slightly apart, and feet flat on floor. (Look at entire body for movements while patient is in this position.) 5) Ask patient to sit with hands hanging unsupported: [for a male patient, hands hanging between his legs, and for a female patient wearing dress, hands hanging over her knees].b (Observe hands and other body areas.) 6) Ask patient to open mouth. (Observe tongue at rest within mouth.) Do this twice. 7) Ask patient to protrude tongue. (Observe abnormalities of tongue movement.) Do this twice. 8) Ask patient to tap thumb, with each finger, as rapidly as possible for 10-15 seconds, separately with right hand, then with left hand. (Observe facial and leg movements.) 9) Flex and extend patient’s left and right arms (one at a time). (Note any rigidity.) 10) Ask patient to stand up. (Observe in profile. Observe all body areas again, hips included.) 11) Ask patient to extend both arms outstretched in front with palms down. (Observe trunk, legs, and mouth.) 12) Have patient walk a few paces, turn, and walk back to chair. (Observe hands and gait.) Do this twice. Movement Ratingsa,b

Complete EXAMINATION PROCEDURE (above) before making ratings for the MOVEMENT RATINGS, rate the highest severity observed.

0  none 1  minimal (may be extreme normal) 2  mild 3  moderate 4  severe

FACIAL & ORAL MOVEMENTS: 1 Muscles of facial expression (e.g., movements of forehead, eyebrows, periorbital area, cheeks; include frowning, blinking, smiling, grimacing [of upper face].) 2 Lips and perioral area (e.g., puckering, pouting, smacking) 3 Jaw (e.g., biting, clenching, chewing, mouth opening, lateral movement) 4 Tongue (Rate only increase in movement both in and out of mouth, NOT inability to sustain a movement.) EXTREMITY MOVEMENTS: 5 Upper (arms, wrists, hands, fingers) Include choreic movements, (i.e., rapid, objectively purposeless, irregular, spontaneous), athetoid movements (i.e., slow, irregular, complex, serpentine). Do NOT include tremor (i.e., repetitive, regular rhythmic) 6 Lower (legs, knees, ankles, toes) e.g., lateral knee movement, foot tapping, heel dropping, foot squirming, inversion & eversion of foot) TRUNK MOVEMENTS: 7 Neck, shoulders, hips (e.g., rocking, twisting, squirming, pelvic gyrations) Include diaphragmatic movements. GLOBAL JUDGMENTS: [score based on highest single score on items 1-7 above] 8 Severity of abnormal movement 9 Incapacitation due to abnormal movement 10 Patient’s awareness of abnormal movement (Rate only patient’s report.) 0  no awareness; 1  aware, no distress; 2  aware, mild distress; 3  aware, moderate distress; 4  aware, severe distress. DENTAL STATUS 11 Current problems with teeth and/or dentures 12 Does patient usually wear dentures?

0

1

2

3

4

0 0 0

1 1 1

2 2 2

3 3 3

4 4 4

0

1

2

3

4

0

1

2

3

4

0

1

2

3

4

0 0 0

1 1 1

2 2 2

3 3 3

4 4 4

Rate item 10 according to reported level of distress

No: 0 No: 0

Yes: 1 Yes: 1

a

Munetz and Benjamin (1988) offer a detailed review of the Examination Procedure as well as a detailed proposal for how to score the Movement Ratings. The authors note the recommendation that the AIMS be administered twice per year beginning prior to the start of neuroleptic (antipsychotic) drug therapy (Baldressarini et al, 1980). They also note that although ratings above 0 may suggest tardive dyskinesia, it is important to determine if the abnormal movement is clinically significant. Also, nonmedication causes should be ruled out. The original AIMS scoring directed that movements (re: Examination Procedure items 8, 11, and 12) be scored 1 point less if they did not occur spontaneously. However, Munetz and Benjamin recommend not following this direction. Schooler and Kane (1982) propose the following criteria for a diagnosis of probable tardive dyskinesia: (a) exposure to neuroleptics for a total of at least 3 months, (b) score of 2 for at least 2 body areas, or score of 3 for at least 1 body area, and (c) rule out other causes for abnormal movement. b AIMS text in brackets was added or rephrased by Munetz and Benjamin (1988). From Guy W: ECDEU Assessment Manual for Psychopharmacology, revised edition, Washington, DC, 1976, US Department of Health, Education, and Welfare. Modified by Munetz MR, Benjamin S: How to examine patients using the Abnormal Involuntary Movement Scale, Hospital Com Psychiatry 39(11):1172–1177, 1988.

525

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Falls Berg Balance Scale Name: __________________________________________ Date:

__________________________________________

General Instructions Please demonstrate each task and/or give instructions as written below. When scoring, please record the lowest response category that applies for each item. In most items, the subject is asked to maintain a given position for a specific time. Progressively more points are deducted if the time or distance requirements are not met, if the subject’s performance warrants supervision, or if the subject touches an external support or receives assistance from the examiner. Subjects should understand that they must maintain their balance while attempting the tasks. The choices of which leg to stand on or how far to reach are left to the subject. Poor judgment will adversely influence the performance and the scoring. Equipment required for testing are a stopwatch or watch with a second hand and a ruler or other indicator of 2, 5, and 10 inches (5, 12.5, and 25 cm). Chairs used during testing should be of reasonable height. Either a step or a stool (of average step height) may be used for item #12.

Item

Description

Score (0-4)

1.

Sitting to standing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

2.

Standing unsupported . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

3.

Sitting unsupported . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

4.

Standing to sitting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

5.

Transfers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

6.

Standing with eyes closed . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

7.

Standing with feet together . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

8.

Reaching forward with outstretched arm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

9.

Retrieving object from floor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

10.

Turning to look behind . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

11.

Turning 360 degrees . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

12.

Placing alternate foot on stool . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

13.

Standing with one foot in front . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________

14.

Standing on one foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ____________ Total ____________

1. Sitting to Standing INSTRUCTIONS: Please stand up. Try not to use your hands for support. ( )4 ( ( ( (

)3 )2 )1 )0

able to stand without using hands and stabilize independently able to stand independently using hands able to stand using hands after several tries needs minimal aid to stand or to stabilize needs moderate or maximal assist to stand

2. Standing Unsupported INSTRUCTIONS: Please stand for two minutes without holding. ( ( ( ( (

)4 )3 )2 )1 )0

able to stand safely 2 minutes able to stand 2 minutes with supervision able to stand 30 seconds unsupported needs several tries to stand 30 seconds unsupported unable to stand 30 seconds unassisted

If a subject is able to stand 2 minutes unsupported, score full points for sitting unsupported. Proceed to item #4.

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Falls Berg Balance Scale (Continued) 3. Sitting with Back Unsupported But Feet Supported on Floor or on a Stool INSTRUCTIONS: Please sit with arms folded for 2 minutes. ( ( ( ( (

)4 )3 )2 )1 )0

able to sit safely and securely 2 minutes able to sit 2 minutes under supervision able to sit 30 seconds able to sit 10 seconds unable to sit without support 10 seconds

4. Standing to Sitting INSTRUCTIONS: Please sit down. ( ( ( ( (

)4 )3 )2 )1 )0

sits safely with minimal use of hands controls descent by using hands uses back of legs against chair to control descent sits independently but has uncontrolled descent needs assistance to sit

5. Transfers INSTRUCTIONS: Arrange chair(s) for a pivot transfer. Ask subject to transfer one way toward a seat with armrests and one way toward a seat without armrests. You may use two chairs (one with and one without armrests) or a bed and a chair. ( ( ( ( (

)4 )3 )2 )1 )0

able to transfer safely with minor use of hands able to transfer safely; definite need of hands able to transfer with verbal cueing and/or supervision needs one person to assist needs two people to assist or supervise to be safe

6. Standing Unsupported with Eyes Closed INSTRUCTIONS: Please close your eyes and stand still for 10 seconds. ( ( ( ( (

)4 )3 )2 )1 )0

able to stand 10 seconds safely able to stand 10 seconds with supervision able to stand 3 seconds unable to keep eyes closed 3 seconds but stays steady needs help to keep from falling

7. Standing Unsupported with Feet Together INSTRUCTIONS: Place your feet together and stand without holding. ( )4 ( )3 ( )2 ( )1 ( )0

able to place feet together independently and stand for 1 minute safely able to place feet together independently and stand for 1 minute with supervision able to place feet together independently and to hold for 30 seconds needs help to attain position but able to stand for 15 seconds with feet together needs help to attain position and unable to hold for 15 seconds

8. Reaching Forward with Outstretched Arm While Standing INSTRUCTIONS: Lift arm to 90 degrees. Stretch out your fingers and reach forward as far as you can. (Examiner places a ruler at end of fingertips when arm is at 90 degrees. Fingers should not touch the ruler while subject is reaching forward. The recorded measure is the distance forward that the fingers reach while the subject is in the most forward leaning position. When possible, ask subject to use both arms when reaching to avoid rotation of the trunk.) ( ( ( ( (

)4 )3 )2 )1 )0

can reach forward confidently 25 cm (10 inches) can reach forward 12.5 cm safely (5 inches) can reach forward 5 cm safely (2 inches) reaches forward but needs supervision loses balance while trying/requires external support

9. Pick Up Object from the Floor from a Standing Position INSTRUCTIONS: Pick up the shoe/slipper that is placed in front of your feet. ( )4 able to pick up slipper safely and easily ( )3 able to pick up slipper but needs supervision

( )2 ( )1 ( )0

unable to pick up slipper but reaches 2-5 cm (1-2 inches) from slipper and keeps balance independently unable to pick up slipper and needs supervision while trying unable to try/needs assist to keep from losing balance or falling

10. Turning to Look Behind Over Left and Right Shoulders While Standing INSTRUCTIONS: Turn to look directly behind you over toward left shoulder. Repeat to the right. Examiner may pick an object to look at directly behind the subject to encourage a better twist turn. ( )4 ( )3 ( )2 ( )1 ( )0

looks behind from both sides and weight shifts well looks behind one side only; other side shows less weight shift turns sideways only but maintains balance needs supervision when turning needs assist to keep from losing balance or falling

11. Turn 360 Degrees INSTRUCTIONS: Turn completely around in a full circle. Pause. Then turn a full circle in the other direction. ( )4 ( )3 ( )2 ( )1 ( )0

able to turn 360 degrees safely in 4 seconds or less able to turn 360 degrees safely one side only in 4 seconds or less able to turn 360 degrees safely but slowly needs close supervision or verbal cueing needs assistance while turning

12. Placing Alternate Foot on Step or Stool While Standing Unsupported INSTRUCTIONS: Place each foot alternately on the step/stool. Continue until each foot has touched the step/stool four times. ( )4 ( )3 ( )2 ( )1 ( )0

able to stand independently and safely and complete 8 steps in 20 seconds able to stand independently and complete 8 steps >20 seconds able to complete 4 steps without aid with supervision able to complete >2 steps; needs minimal assist needs assistance to keep from falling/unable to try

13. Standing Unsupported One Foot in Front INSTRUCTIONS: (Demonstrate to Subject) Place one foot directly in front of the other. If you feel that you cannot place your foot directly in front, try to step far enough ahead that the heel of your forward foot is ahead of the toes of the other foot. (To score 3 points, the length of the step should exceed the length of the other foot and the width of the stance should approximate the subject’s normal stride width) ( )4 ( )3 ( )2 ( )1 ( )0

able to place foot tandem independently and hold 30 seconds able to place foot ahead of other independently and hold 30 seconds able to take small step independently and hold 30 seconds needs help to step but can hold 15 seconds loses balance while stepping or standing

14. Standing on One Leg INSTRUCTIONS: Stand on one leg as long as you can without holding. ( ( ( (

)4 )3 )2 )1

( )0

able to lift leg independently and hold 10 seconds able to lift leg independently and hold 5-10 seconds able to lift leg independently and hold  or 3 seconds tries to lift leg; unable to hold 3 seconds but remains standing independently unable to try or needs assist to prevent fall

( ) Total Score (Maximum  56)

527

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528

Falls Timed Up and Go Test Targeted Population: Older adults. Description: The timed Up and Go test is a modified, timed version of the Get Up and Go test (Mathias et al, 1986), which evaluates gait and balance. The patient is observed and timed while rising from an armchair, walking 3 meters, turning, walking back, and sitting down again. No physical assistance is given. The patient wears regular footwear and uses any customary walking aid. A stopwatch or wristwatch with a second hand is used to time this activity. The patient is allowed one practice trial and then three actual trials. The times from the three actual trials are averaged. This test is quick, requires no special equipment or training, and is easily included as part of the routine medical examination. Scores: A score of  10 seconds  freely mobile,  20 seconds  mostly independent, 20 to 29 seconds  variable mobility,  30 seconds  impaired mobility. A score of 30 seconds or greater indicates that the patient has impaired mobility, requires assistance, and is at a high risk for falls. Accuracy: The authors reported that the time score was reliable (interrater and intrarater); correlated well with log-transformed scores on the Berg Balance Scale, gait speed, and Barthel Index of ADL; and appeared to predict the patient’s ability to go outside alone safely. The authors suggest that the timed Up and Go test is a reliable and valid test for quantifying functional mobility, which may also be useful in monitoring clinical change over time. Administration Time:  2 minutes. From Podsiadlo D, Richardson S: The timed “Up and Go:” a test of basic functional mobility for frail, elderly persons, J Am Geriatric Soc 39:142, 1991. Reprinted with permission Blackwell Publishing Ltd. Descriptive summary from Loretz L: Primary Care Tools for Clinicians: A Compendium of Forms, Questionnaires, and Rating Scales for Everyday Practice, St. Louis, 2005, Mosby.

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Falls Tinetti Balance and Gait Evaluation Performance-Oriented Assessment of Mobility Name: __________________________________________ Date:

__________________________________________

Address: ________________________________________ Phone: __________________________________________ D.O.B.: ___________________ Medical Record #:

Gender: Male

Female

________________________________

Balance Instructions: Subject is seated in a hard, armless chair. The following maneuvers are tested:

1. Sitting balance 0  Leans or slides in chair 1  Steady, safe 2. Arise 0  Unable without help 1  Able but uses arm to help 2  Able without use of arms 3. Attempts to arise 0  Unable without help 1  Able, but requires more than one attempt 2  Able to arise with one attempt 4. Immediate standing balance (first 5 seconds) 0  Unsteady (staggers, moves feet, marked trunk sway) 1  Steady but uses walker/cane or grabs other object for support 2  Steady without walker or cane or other support 5. Standing balance 0  Unsteady 1  Steady, but wide stance (medial heels > than 4 apart) or uses cane/walker or other support 2  Narrow stance without support 6. Nudge (subject at maximum position with feet as close together as possible. Examiner pushes lightly on subject’s sternum with palm of hand 3 times.) 0  Begins to fall 1  Staggers, grabs, but catches self 2  Steady 7. Eyes closed (at maximum position #6) 0  Unsteady 1  Steady 8. Turn 360° 0  Discontinuous steps 1  Continuous steps 0  Unsteady (grabs, staggers) 1  Steady 9. Sit down 0  Unsafe (misjudged distance; falls into chair) 1  Uses arms or not a smooth motion 2  Safe, smooth motion

/16

BALANCE SCORE Continued on following page

529

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530

Falls Tinetti Balance and Gait Evaluation (Continued) Gait Instructions: Subject stands with examiner. Walks down hallway or across room, first at his/her usual pace, then back at a “rapid but safe” pace (using usual walking aid such as cane/walker).

10. Initiation of gait (immediately after told “go”) 0  Any hesitancy or multiple attempts to start 1  No hesitancy 11. Step length and height (right foot swing) 0  Does not pass L. stance foot with step 1  Passes L. stance foot 0  R. foot does not clear floor completely with step 1  R. foot completely clears floor 12. Step length and height (left foot swing) 0  Does not pass R. stance foot with step 1  Passes R. stance foot 0  L. foot does not clear floor completely with step 1  L. foot completely clears floor 13. Step symmetry 0  R. and L. step length not equal (estimate) 1  R. and L. step length appear equal 14. Step continuity 0  Stopping or discontinuity between steps 1  Steps appear continuous 15. Path (estimated in relation to floor tiles, 12” wide. Observe excursion of one foot over about 10 feet of course.) 0  Marked deviation 1  Mild/moderate deviation or uses a walking aid 2  Straight without walking aid 16. Trunk 0  Marked sway or uses walking aid 1  No sway but flexion of knees or back or spreads arms out while walking 2  No sway, no flexion, no use of arms and no walking aid 17. Walk stance 0  Heels apart 1  Heels almost touching while walking /12

GAIT SCORE

/28

TOTAL MOBILITY SCORE (BALANCE AND GAIT)

From Tinetti ME: Performance-oriented assessment of mobility problems in elderly patients, J Am Geriatric Soc 34:119–126, 1986. Reprinted with permission Blackwell Publishing, Ltd.

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Hearing Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S)* Name: __________________________________________ Date:

__________________________________________

Address: ________________________________________ Phone: __________________________________________ D.O.B.: ___________________ Medical Record #:

Gender: Male

Female

________________________________

Yes (4)

Sometimes (2)

No (0)

E-1. Does a hearing problem cause you to feel embarrassed when meeting new people?

________

________

________

E-2. Does a hearing problem cause you to feel frustrated when talking to members of your family?

________

________

________

S-3. Do you have difficulty hearing when someone speaks in a whisper?

________

________

________

E-4. Do you feel handicapped by a hearing problem?

________

________

________

S-5. Does a hearing problem cause difficulty when visiting friends, relatives, or neighbors?

________

________

________

S-6. Does a hearing problem cause you to attend religious services less often than you would like?

________

________

________

E-7. Does a hearing problem cause you to have arguments with family members?

________

________

________

S-8. Does a hearing problem cause you difficulty when listening to TV or radio?

________

________

________

________

________

________

________

________

________

E-9. Do you feel that any difficulty with your hearing limits or hampers your personal or social life? S-10. Does a hearing problem cause you difficulty when in a restaurant with relatives or friends?

E  emotional response S  social/situational response *Range of total points, 0-40; 0-8, no self-perceived handicap; 10-22, mild to moderate handicap; 24-40, significant handicap From Ventry IM, Weinstein BE: Identification of elderly people with hearing problems, ASHA July 25:37, 1983. Copyright by the American-Speech-Language-Hearing Association. Reprinted with permission.

531

35

36

37

38

144 151 159 166 174 182 189 197 204 212 219 227 235 242 250 257 265 272 280 288 295 302 310 318 325 333 340 348 355 363 371 378 386 393 401 408

148 155 163 171 179 186 194 202 210 218 225 233 241 249 256 264 272 280 287 295 303 311 319 326 334 342 350 358 365 373 381 389 396 404 412 420

152 160 168 176 184 192 200 208 216 224 232 240 248 256 264 272 279 287 295 303 311 319 327 335 343 351 359 367 375 383 391 399 407 415 423 431

156 164 172 180 189 197 205 213 221 230 238 246 254 263 271 279 287 295 304 312 320 328 336 344 353 361 369 377 385 394 402 410 418 426 435 443

74

75

76

Adapted from Clinical Guidelines on the Identification, Evaluation, and Treatment of Overweight and Obesity in Adults: The Evidence Report.

99 104 109 114 119 124 128 133 138 143 148 153 158 163 168 173 178 183 188 193 198 203 208 212 217 222 227 232 237 242 247 252 257 262 267

73

54

140 147 154 162 169 177 184 191 199 206 213 221 228 235 242 250 258 265 272 279 287 294 302 309 316 324 331 338 346 353 361 368 375 383 390 397

53

72

52

136 143 150 157 165 172 179 186 193 200 208 215 222 229 236 243 250 257 265 272 279 286 293 301 308 315 322 329 338 343 351 358 365 372 379 386

51

71

50

132 139 146 153 160 167 174 181 188 195 202 209 216 222 229 236 243 250 257 264 271 278 285 292 299 306 313 320 327 334 341 348 355 362 369 376

49

70

48

128 135 142 149 155 162 169 176 182 189 196 203 209 216 223 230 236 243 250 257 263 270 277 284 291 297 304 311 318 324 331 338 345 351 358 365

47

69

46

125 131 138 144 151 158 164 171 177 184 190 197 203 210 216 223 230 236 243 249 256 262 269 276 282 289 295 302 308 315 322 328 335 341 348 354

45

68

44

121 127 134 140 146 153 159 166 172 178 185 191 198 204 211 217 223 230 236 242 249 255 261 268 274 280 287 293 299 306 312 319 325 331 338 344

42 43

67

41

118 124 130 136 142 148 155 161 167 173 179 186 192 198 204 210 216 223 229 235 241 247 253 260 266 272 278 284 291 297 303 309 315 322 328 334

40

66

39

114 120 126 132 138 144 150 156 162 168 174 180 186 192 198 204 210 216 222 228 234 240 246 252 258 264 270 276 282 288 294 300 306 312 318 324

Body Weight (pounds)

34

65

33

110 116 122 128 134 140 145 151 157 163 169 174 180 186 192 197 204 209 215 221 227 232 238 244 250 256 262 267 273 279 285 291 296 302 308 314

32

64

31

107 113 118 124 130 135 141 146 152 158 163 169 175 180 186 191 197 203 208 214 220 225 231 237 242 248 254 259 265 270 278 282 287 293 299 304

30

63

29

104 109 115 120 126 131 136 142 147 153 158 164 169 175 180 186 191 196 202 207 213 218 224 229 235 240 246 251 256 262 267 273 278 284 289 295

28

62

27

100 106 111 116 122 127 132 137 143 148 153 158 164 169 174 180 185 190 195 201 206 211 217 222 227 232 238 243 248 254 259 264 269 275 280 285

26

61

25

97 102 107 112 118 123 128 133 138 143 148 153 158 163 168 174 179 184 189 194 199 204 209 215 220 225 230 235 240 245 250 255 261 266 271 276

24

60

23

94

22

59

21

Extreme Obesity

96 100 105 110 115 119 124 129 134 138 143 148 153 158 162 167 172 177 181 186 191 196 201 205 210 215 220 224 229 234 239 244 248 253 258

20

Obese

91

19

Overweight

58

Height (inches)

BMI

Normal

532

Body Mass Index

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Nutrition

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Nutrition

Copyright Nestle, 1994, Revision 1998, Societe des Produits Nestle S.A.

533

20 19 18 17 16 15 14 13 12 11 10 9 8 7 6 5 4 3 2 1 0

________ No pain

________ Slight pain

________ Mild pain

________ Moderate pain

________ Severe pain

________ Very severe pain

________ The most intense pain imaginable

Verbal Descriptor Scale (VDS)

No Pain

The Worst Imaginable Pain

Visual Analog Scale

534

Numeric Rating Scale (NRS)

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Pain

Pain Assessment

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Physical Function Instrumental Activities of Daily Living Scale (IADL) Name: __________________________________________ Date:

__________________________________________

D.O.B.: _____________________Gender: Male

Female

Medical Record #: ________________________________

Circle one statement in each category A-H that applies to subject. E. Laundry

A. Ability to Use Telephone 1. Operates telephone on own initiative; looks up and dials numbers, etc.

1

2. Dials a few well-known numbers.

1

3. Answers telephone but does not dial.

1

4. Does not use telephone at all.

0

B. Shopping

1. Does personal laundry completely.

1

2. Launders small items; rinses stockings, etc.

1

3. All laundry must be done by others.

0

F. Mode of Transportation 1. Travels independently on public transportation or drives own car.

1

1. Takes care of all shopping needs independently

1

2. Arranges own travel via taxi, but does not otherwise use public transportation.

1

2. Shops independently for small purchases

0

1

3. Needs to be accompanied on any shopping trip.

0

3. Travels on public transportation when accompanied by another.

0

4. Completely unable to shop.

0

4. Travel limited to taxi or automobile with assistance of another. 5. Does not travel at all.

0

C. Food Preparation 1. Plans, prepares, and serves adequate meals independently.

1

2. Prepares adequate meals if supplied with ingredients.

0

3. Heats, serves, and prepares meals or prepares meals but does not maintain adequate diet.

0

4. Needs to have meals prepared and served.

0

D. Housekeeping 1. Maintains house alone or with occasional assistance (e.g., “heavy work domestic help”).

1

2. Performs light daily tasks such as dishwashing, bed making.

1

3. Performs light daily tasks but cannot maintain acceptable level of cleanliness.

1

4. Needs help with all home maintenance tasks.

1

5. Does not participate in any housekeeping tasks.

0

G. Responsibility for Own Medications 1. Is responsible for taking medication in correct dosages at correct time.

1

2. Takes responsibility if medication is prepared in advance in separate dosage.

0

3. Is not capable of dispensing own medication.

0

H. Ability to Handle Finances 1. Manages financial matters independently (budgets, writes checks, pays rent and bills, goes to bank), collects and keeps track of income.

1

2. Manages day-to-day purchases, but needs help with banking, major purchases, etc.

1

3. Incapable of handling money.

0

OVERALL SCORE: 7-8  high level independence 5-6  moderate level independence 3-4  moderate level dependence 1-2  dependence

Adapted from Lawton MP, Brody EM: Assessment of older people: self-maintaining and instrumental activities of daily living, Gerontologist 9:179–186, 1969.

535

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536

Physical Function Katz Index of Activities of Daily Living

From Katz S, Downs TD, Cash HR, et al: Progress in the development of the Index of ADL, Gerontologist 10:23, 1970.

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Physical Function The Index of Independence in Activities of Daily Living: Scoring and Definitions (Katz)

537

538

Preoperative Cardiac Risk Assessment Stepwise Approach to Preoperative Cardiac Assessment

From ACC/AHA Guidelines for the Perioperative Cardiovascular Evaluation for Noncardiac Surgery, J Am Coll Cardiol 27:910–948, 1996. Copyright 2002 by the American College of Cardiology and the American Heart Association, Inc. Reprinted with permission.

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Preoperative Cardiac Risk Assessment Indications for Coronary Angiography* in Perioperative Evaluation Before (or After) Noncardiac Surgery

TABLE A2-1

Class I†: Patients with suspected or proven CAD: • High-risk results during noninvasive testing • Angina pectoris unresponsive to adequate medical therapy • Most patients with unstable angina pectoris • Nondiagnostic or equivocal noninvasive test in a high-risk patient (Table A2-3) undergoing a high-risk noncardiac surgical procedure (Table A2-5) Class II†: • Intermediate-risk results during noninvasive testing • Nondiagnostic or equivocal noninvasive test in a lower-risk patient (Table A2-3) undergoing a high-risk noncardiac surgical procedure (Table A2-5) • Urgent noncardiac surgery in a patient convalescing from acute MI • Perioperative MI Class III†: • Low-risk noncardiac surgery (Table A2-5) in a patient with known CAD and low-risk results on noninvasive testing • Screening for CAD without appropriate noninvasive testing • Asymptomatic after coronary revascularization, with excellent exercise capacity (7 METs) • Mild stable angina in patients with good LV function, low-risk noninvasive test results • Patient is not a candidate for coronary revascularization because of concomitant medical illness • Prior technically adequate normal coronary angiogram within 5 years • Severe LV dysfunction (eg, ejection fraction 20%) and patient not considered candidate for revascularization procedure • Patient unwilling to consider coronary revascularization procedure *If results will affect management. †Class I: Conditions for which there is evidence for and/or general agreement that a procedure be performed or a treatment is of benefit. Class II: Conditions for which there is a divergence of evidence and/or opinion about the treatment. Class III: Conditions for which there is evidence and/or general agreement that the procedure is not necessary. CAD, Coronary artery disease; LV, left ventricular; MET, metabolic equivalent; MI, myocardial infarction. Adapted from ACC/AHA Guidelines for Coronary Angiography.

Prognostic Gradient of Ischemic Responses During an ECG-Monitored Exercise Test

TABLE A2-2

Patients with suspected or proven CAD High Risk Ischemia induced by low-level exercise* (4 METs or heart rate 100 bpm or 70% age predicted) manifested by one or more of the following: • Horizontal or downsloping ST depression 0.1 mV • ST-segment elevation 0.1 mV in noninfarct lead • Five or more abnormal leads • Persistent ischemic response 3 min after exertion • Typical angina Intermediate Risk Ischemia induced by moderate-level exercise (4-6 METs or heart rate 100-130 bpm [70-85% age predicted]) manifested by one or more of the following: • Horizontal or downsloping ST depression 0.1 mV • Typical angina • Persistent ischemic response 1-3 min after exertion • Three to four abnormal leads Low Risk No ischemia or ischemia induced at high-level exercise (7 METs or heart rate 130 bpm [85% age predicted]) manifested by: • Horizontal or downsloping ST depression 0.1 mV • Typical angina • One to two abnormal leads Inadequate Test Inability to reach adequate target workload or heart rate response for age without an ischemic response. For patients undergoing noncardiac surgery, ability to exercise to at least the intermediate-risk level without ischemia should be considered at low risk for perioperative ischemic events. Bpm, Beats per minute; ECG, electrocardiographically; MET, metabolic equivalent. *Workload and heart rate estimates for risk severity require adjustment for patient age. Maximum target heart rates for 40- and 80-year-old subjects on no cardioactive medication are 180 and 140 beats per minute, respectively.

Clinical Predictors of Increased Perioperative Cardiovascular Risk (Myocardial Infarction, Congestive Heart Failure, Death)

TABLE A2-3

Major Unstable coronary syndromes • Recent myocardial infarction* with evidence of important ischemic risk by clinical symptoms or noninvasive study • Unstable or severe† angina (Canadian Class III or IV)‡ Decompensated congestive heart failure Significant arrhythmias • High-grade atrioventricular block • Symptomatic ventricular arrhythmias in the presence of underlying heart disease • Supraventricular arrhythmias with uncontrolled ventricular rate Severe valvular disease Intermediate Mild angina pectoris (Canadian Class I or II) Prior myocardial infarction by history or pathological Q waves Compensated or prior congestive heart failure Diabetes mellitus Minor Advanced age Abnormal ECG (left ventricular hypertrophy, left bundle branch block, ST-T abnormalities) Rhythm other than sinus (eg, atrial fibrillation) Low functional capacity (eg, inability to climb one flight of stairs with a bag of groceries) History of stroke Uncontrolled systemic hypertension ECG, Electrocardiogram. *The American College of Cardiology National Database Library defines recent MI as greater than 7 days but less than or equal to 1 month (30 days). †May include “stable” angina in patients who are unusually sedentary. ‡Campeau L. Grading of angina pectoris. Circulation. 1976;54:522-523.

539

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Preoperative Cardiac Risk Assessment TABLE A2-4 1 MET

Estimated Energy Requirements for Various Activities

Can you take care of yourself? Eat, dress, or use the toilet? Walk indoors around the house? Walk a block or two on level ground at 2-3 mph or 3.2-4.8 km/h? Do light work around the house like dusting or washing dishes?

4 METs

Climb a flight of stairs or walk up a hill? Walk on level ground at 4 mph or 6.4 km/h? Run a short distance? Do heavy work around the house like scrubbing floors or lifting or moving heavy furniture? Participate in moderate recreational activities like golf, bowling, dancing, doubles tennis, or throwing a baseball or football?

10 METs

Participate in strenuous sports like swimming, singles tennis, football, basketball, or skiing?

4 METs

Cardiac Risk* Stratification for Noncardiac Surgical Procedures

TABLE A2-5 High

Intermediate

Low†

(Reported cardiac risk often 5%) • Emergent major operations, particularly in the elderly • Aortic and other major vascular • Peripheral vascular • Anticipated prolonged surgical procedures associated with large fluid shifts and/or blood loss (Reported cardiac risk generally 5%) • Carotid endarterectomy • Head and neck • Intraperitoneal and intrathoracic • Orthopedic • Prostate (Reported cardiac risk generally 1%) • Endoscopic procedures • Superficial procedure • Cataract • Breast

*Combined incidence of cardiac death and nonfatal myocardial infarction. †Do not generally require further preoperative cardiac testing. From ACC/AHA Guidelines for the Perioperative Cardiovascular Evaluation for Noncardiac Surgery, J Am Coll Cardiol 27:910–948, 1996. Copyright 2002 by the American College of Cardiology and the American Heart Association, Inc. Reprinted with permission.

© Copyright

1. Problem Requires moderate to maximum assistance in moving. Complete lifting without sliding against sheets is impossible. Frequently slides down in bed or chair, requiring frequent repositioning with maximum assistance. Spasticity, contractures, or agitation leads to almost constant friction.

1. Very Poor Never eats a complete meal. Rarely eats more than 1–3 of any food offered. Eats 2 servings or less of protein (meat or dairy products) per day. Takes fluids poorly. Does not take a liquid dietary supplement OR is NPO and/or maintained on clear liquids or IV’s for more than 5 days.

1. Completely Immobile Does not make even slight changes in body or extremity position without assistance.

1. Bedfast Confined to bed.

1. Constantly Moist Skin is kept moist almost constantly by perspiration, urine, etc. Dampness is detected every time patient is moved or turned.

1. Completely Limited Unresponsive (does not moan, flinch, or grasp) to painful stimuli, due to diminished level of consciousness or sedation. OR limited ability to feel pain over most of body.

2. Potential Problem Moves feebly or requires minimum assistance. During a move skin probably slides to some extent against sheets, chair, restraints, or other devices. Maintains relatively good position in chair or bed most of the time but occasionally slides down.

2. Probably Inadequate Rarely eats a complete meal and generally eats only about 1–2 of any food offered. Protein intake includes only 3 servings of meat or dairy products per day. Occasionally will take a dietary supplement. OR receives less than optimum amount of liquid diet or tube feeding.

2. Very Limited Makes occasional slight changes in body or extremity position but unable to make frequent or significant changes independently.

2. Chairfast Ability to walk severely limited or non-existent. Cannot bear own weight and/or must be assisted into chair or wheelchair.

2. Very Moist Skin is often, but not always, moist. Linen must be changed at least once a shift.

2. Very Limited Responds only to painful stimuli. Cannot communicate discomfort except by moaning or restlessness. OR has a sensory impairment which limits the ability to feel pain or discomfort over 1–2 of body.

3. No Apparent Problem Moves in bed and in chair independently and has sufficient muscle strength to lift up completely during move. Maintains good position in bed or chair.

3. Adequate Eats over half of most meals. Eats a total of 4 servings of protein (meat, dairy products) per day. Occasionally will refuse a meal, but will usually take a supplement when offered. OR is on a tube feeding or TPN regimen which probably meets most of nutritional needs.

3. Slightly Limited Makes frequent though slight changes in body or extremity position independently.

3. Walks Occasionally Walks occasionally during day, but for very short distances, with or without assistance. Spends majority of each shift in bed or chair.

3. Occasionally Moist Skin is occasionally moist, requiring an extra linen change approximately once a day.

3. Slightly Limited Responds to verbal commands, but cannot always communicate discomfort or the need to be turned. OR has some sensory impairment which limits ability to feel pain or discomfort in 1 or 2 extremities.

Evaluator’s Name________________________________

Barbara Braden and Nancy Bergstrom, 1988. Reprinted with permission.

FRICTION & SHEAR

usual food intake pattern

NUTRITION

ability to change and control body position

MOBILITY

degree of physical activity

ACTIVITY

exposed to moisture

MOISTURE

ability to respond meaningfully to pressure-related discomfort

SENSORY PERCEPTION

Patient’s Name _____________________________________

Braden Scale for Predicting Pressure Sore Risk Date of Assessment

Total Score

4. Excellent Eats most of every meal. Never refuses a meal. Usually eats a total of 4 or more servings of meat and dairy products. Occasionally eats between meals. Does not require supplementation.

4. No Limitation Makes major and frequent changes in position without assistance.

4. Walks Frequently Walks outside room at least twice a day and inside room at least once every two hours during waking hours.

4. Rarely Moist Skin is usually dry, linen only requires changing at routine intervals.

4. No Impairment Responds to verbal commands. Has no sensory deficit which would limit ability to feel or voice pain or discomfort.

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Pressure Ulcer 541

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Pressure Ulcer National Pressure Ulcer Advisory Panel (NPUAP) Staging System for Pressure Ulcerations Stage I: Nonblanchable erythema of intact skin, the heralding lesion of skin ulceration. In individuals with darker skin, discoloration of the skin, warmth, edema, induration, or hardness may also be indicators. Stage II: Partial-thickness skin loss involving epidermis, dermis, or both. The ulcer is superficial and presents clinically as an abrasion, blister, or shallow crater. Stage III: Full-thickness skin loss involving damage to or necrosis of subcutaneous tissue that may extend down to, but not through, underlying fascia. The ulcer presents clinically as a deep crater with or without undermining of adjacent tissue. Stage IV: Full-thickness skin loss with extensive destruction, tissue necrosis, or damage to muscle, bone, or supporting structures (e.g., tendon, joint capsule). Undermining and sinus tracts may also be associated with Stage IV pressure ulcers. National Pressure Ulcer Advisory Panel, 1998. website: http://www.npuap.org

Date

3 2 1

Fair Poor Very bad

Stupor

Confused

Apathetic

Alert

Mental condition

1

2

3

4

Stupor

Chairbound

Walk/help

Ambulant

Activity

1

2

3

4

Immobile

Very limited

Slightly limited

Full

Mobility

1

2

3

4

Doubly

Usually/Urine

Occasional

Not

Incontinent

1

2

3

4 Total score

From Norton D, McLaren R, Exton-Smith AN: An investigation of geriatric nursing problems in hospitals, 1962. Copyright National Corporation for the Care of Old People (now Centre for Policy on Ageing), London, England. Reprinted with permission.

Interpretation: maximum score 20; minimum score 5; at risk for pressure ulcer if score 14.

Name

4

Good

Physical condition

Norton Pressure Ulcer Prediction Scale

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Pressure Ulcer 543

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Pressure Ulcer

Pressure Ulcer Scale for Healing (PUSH)

PUSH Tool 3.0 Patient Name________________________________________________ Patient ID# _____________________ Ulcer Location ____________________________________________________ Date _____________________

Directions: Observe and measure the pressure ulcer. Categorize the ulcer with respect to surface area, exudate, and type of wound tissue. Record a sub-score for each of these ulcer characteristics. Add the sub-scores to obtain the total score. A comparison of total scores measured over time provides an indication of the improvement or deterioration in pressure ulcer healing.

LENGTH X WIDTH (in

0

1

2

3

4

5

0

< 0.3

0.3 – 0.6

0.7 – 1.0

1.1 – 2.0

2.1 – 3.0

6

7

8

9

10

3.1 – 4.0

4.1 – 8.0

8.1 – 12.0

12.1 – 24.0

> 24.0

0

1

2

3

None

Light

Moderate

Heavy

3

4

Slough

Necrotic Tissue

Sub-score

cm2)

EXUDATE AMOUNT TISSUE TYPE

0

1

2

Closed

Epithelial Tissue

Granulation Tissue

Sub-score

Sub-score

TOTAL SCORE

Length x Width: Measure the greatest length (head to toe) and the greatest width (side to side) using a centimeter ruler. Multiply these two measurements (length x width) to obtain an estimate of surface area in square centimeters (cm2). Caveat: Do not guess! Always use a centimeter ruler and always use the same method each time the ulcer is measured. Exudate Amount: Estimate the amount of exudate (drainage) present after removal of the dressing and before applying any topical agent to the ulcer. Estimate the exudate (drainage) as none, light, moderate, or heavy. Tissue Type: This refers to the types of tissue that are present in the wound (ulcer) bed. Score as a “4” if there is any necrotic tissue present. Score as a “3” if there is any amount of slough present and necrotic tissue is absent. Score as a “2” if the wound is clean and contains granulation tissue. A superficial wound that is reepithelializing is scored as a “1”. When the wound is closed, score as a “0”. 4 – Necrotic Tissue (Eschar): black, brown, or tan tissue that adheres firmly to the wound bed or ulcer edges and may be either firmer or softer than surrounding skin. 3 – Slough: yellow or white tissue that adheres to the ulcer bed in strings or thick clumps, or is mucinous. 2 – Granulation Tissue: pink or beefy red tissue with a shiny, moist, granular appearance. 1 – Epithelial Tissue: for superficial ulcers, new pink or shiny tissue (skin) that grows in from the edges or as islands on the ulcer surface. 0 – Closed/Resurfaced: the wound is completely covered with epithelium (new skin). ©National Pressure Ulcer Advisory Panel Reprinted with permission.

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Pressure Ulcer

Pressure Ulcer Scale for Healing (PUSH)

PUSH Tool 3.0 Patient Name________________________________________________ Patient ID# _____________________ Ulcer Location ____________________________________________________ Date _____________________

Directions: Observe and measure pressure ulcers at regular intervals using the PUSH Tool. Date and record PUSH sub-scores and Total Scores on the Pressure Ulcer Healing Record below.

Pressure Ulcer Healing Record Date Length x Width Exudate Amount Tissue Type PUSH Total Score Graph the PUSH Total Scores on the Pressure Ulcer Healing Graph below. PUSH Total Score

Pressure Ulcer Healing Graph

17 16 15 14 13 12 11 10 9 8 7 6 5 4 3 2 1 Healed = 0 Date ©National Pressure Ulcer Advisory Panel Reprinted with permission.

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Quality of Life Medical Outcomes Study Short Form 36 (MOS SF-36) Targeted Population: Adolescents (age 14 and older) and adults. Description: Developed from the work at the Rand Corporation in Santa Monica, California, the MOS SF-36 measures health status and outcomes from the patient’s point of view. The MOS SF-36 contains 36 items that measure eight dimensions: physical activities (10 items), role limitations caused by physical health problems (4 items), role limitations caused by emotional problems (3 items), social limitations (2 items), pain (2 items), general health perceptions (5 items), vitality and fatigue (4 items), and mental health (5 items). There is one question in which subjects are asked to compare current health to that of one year previous to provide an estimate of change in health status. The MOS SF-36 is one of the most well-known health status instruments and is used around the world. Scores: Each item has a different scoring format, ranging from a simple “yes/no” response to Likert-type scales. An equation with weighted scores is used to obtain item scores; mean scores may be calculated for subscales. The scoring method is complex, is described fully in the user’s manual, and can be done with automated software. An overall score is not reported. Accuracy: The MOS SF-36 and MOS SF-12 are well-developed, concise instruments with excellent core descriptions of reliability and validity (McHorney et al, 1993, 1994). Many studies detailing the good to excellent reliability and validity of these tools are reviewed by McDowell and Newell (1996) and Bowling (1997). The MOS SF-36 has become a standard against which other instruments are measured. Administration Time: 5 to 15 minutes by self-administration or by trained interviewer. Co-copyright and trademark holders for the Medical Outcomes Study Short Form 36 (MOS SF-36) and Medical Outcomes Study Short Form 12 (MOS SF-12) are the Medical Outcomes Trust (MOT), the Health Assessment Lab, and QualityMetric Incorporated. Information regarding licensing, use, and ordering may be found online at http://www.qualitymetric.com/products/descriptions/sflicenses.shtml. The forms may be viewed at http://www.qualitymetric.com/products/sfsurveys.aspx. The MOS SF-36, MOS SF-12, and other quality-of-life forms may also be viewed at http://www.rand.org/health/surveys.html.

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Quality of Life Missoula-VITAS Quality of Life Index (MVQOLI) Improving quality of life for patients is the primary goal for hospice care and for end-of-life care in any setting. The Missoula-VITAS Quality of Life Index (MVQOLI) is specifically designed to evaluate the patient’s experience of quality of life during advanced illness. Based on Dr. Ira Byock’s model of growth and development at the end of life, the MVQOLI is used both as an assessment tool to inform care planning and as an outcome measure. The instrument produces a quality of life profile for each individual patient that graphically reveals the influence of 5 domains of experience on quality of life. The instrument contains 25 items that assess 5 dimensions of a person’s subjective experience—symptoms, functional status, interpersonal relations, emotional well-being, and transcendence. MISSOULA-VITAS QUALITY OF LIFE INDEX (TM) V - 25 INSTRUCTIONS: Indicate the extent to which you agree or disagree with the following statements by marking in one of the circles below the question. For items with two statements, indicate agreement with one or the other or if they are equally true, choose “Neutral.” If you make a mistake or change your mind, place an X through the wrong answer and mark the circle indicating your correct answer. Patient’s Name: _________________ Today’s Date: _________________ GLOBAL How would you rate your overall quality of life?     Worst Poor Fair Good Possible

 Best Possible

SYMPTOM 1. My symptoms are adequately controlled.     Agree Agree Neutral Disagree Strongly

8. I am satisfied with my ability to take care of my basic needs.      Agree Agree Neutral Disagree Disagree Strongly Strongly

 Disagree Strongly

2. I feel sick all the time.   Agree Agree Strongly

9. I accept the fact that I OR I am disappointed that I can not do many of the cannot do many of the things I used to do. things I used to do.      Agree Agree Neutral Disagree Disagree Strongly Strongly

 Disagree Strongly

 Neutral

 Disagree

3. I accept my symptoms as a fact of life.     Agree Agree Neutral Disagree Strongly

 Disagree Strongly

10. My contentment with life depends upon being active and being independent in my personal care.      Agree Agree Neutral Disagree Disagree Strongly Strongly

4. I am satisfied with current control of my symptoms.      Agree Agree Neutral Disagree Disagree Strongly Strongly

INTERPERSONAL 11. I have recently been able to say important things to the people close to me.      Agree Agree Neutral Disagree Disagree Strongly Strongly

5. Despite physical discomfort, in general I can enjoy my days.   Agree Agree Strongly

OR

Physical discomfort overshadows an opportunity for enjoyment.    Neutral Disagree Disagree Strongly

12. I feel closer to others OR I feel increasingly distant in my life now than I from others in my life. did before my illness.      Agree Agree Neutral Disagree Disagree Strongly Strongly

FUNCTION 6. I am still able to attend OR I am dependent on others to most of my personal for my personal care. needs by myself.      Agree Agree Neutral Disagree Disagree Strongly Strongly

13. In general, these days I am satisfied with relationships with family and friends.      Agree Agree Neutral Disagree Disagree Strongly Strongly

7. I am still able to do many of the things I like to do.   Agree Agree Strongly

OR

I am no longer able to do many of the things I like to do.    Neutral Disagree Disagree Strongly

14. At present, I spend as much time as I want to with family and friends.      Agree Agree Neutral Disagree Disagree Strongly Strongly

Continued on following page

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Quality of Life INTERPERSONAL (Continued) 15. It is important to me to have close personal relationships.      Agree Agree Neutral Disagree Disagree Strongly Strongly WELL-BEING 16. My affairs are in order; OR My affairs are not in order; I could die today with I am worried that many a clear mind. things are unresolved.      Agree Agree Neutral Disagree Disagree Strongly Strongly 17. I feel generally at peace and prepared to leave this life.   Agree Agree Strongly

OR

I am unsettled and unprepared to leave this life.    Neutral Disagree Disagree Strongly

18. I am more satisfied with myself as a person now than I was before my illness.      Agree Agree Neutral Disagree Disagree Strongly Strongly 19. The longer I am ill, the more I worry about things “getting out of control.”   Agree Agree Strongly

OR

 Neutral

The longer I am ill, the more comfortable I am with the idea of “letting go.”  Disagree

 Disagree Strongly

20. It is important to me to be at peace with myself.      Agree Agree Neutral Disagree Disagree Strongly Strongly

TRANSCENDENT 21. I have a greater sense OR I feel more disconnected of connection to all from all things now than things now than I did I did before my illness. before my illness.      Agree Agree Neutral Disagree Disagree Strongly Strongly 22. I have a better sense OR I have less of a sense of of meaning in my life meaning in my life now now than I have had than I have had in the past. in the past.      Agree Agree Neutral Disagree Disagree Strongly Strongly 23. As the end of my life OR approaches, I am comfortable with the thought of my own death.    Agree Agree Neutral Strongly

24. Life has become more OR Life has lost all value for precious to me; every me; every day is a burden. day is a gift.      Agree Agree Neutral Disagree Disagree Strongly Strongly 25. It is important to me to feel that my life has meaning.      Agree Agree Neutral Disagree Disagree Strongly Strongly Did you complete this questionnaire by yourself?   Yes No

© 1998 -2001 by VITAS Healthcare Corporation, Miami, Florida, and Ira R. Byock, MD, Missoula, Montana. Used with permission. The form may be viewed online at http://www.dyingwell.com/MVQOLI.htm..

As the end of my life approaches, I am uneasy with the thought of my own death.   Disagree Disagree Strongly

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Quality of Life Nottingham Health Profile (NHP) Targeted Population: Adults. Description: The Nottingham Health Profile (NHP) provides a brief indication of a patient’s perceived emotional, social, and physical health problems (Hunt et al, 1981, 1985). It serves to identify how people feel when they experience ill health. There are 45 items, 38 in Part I and 7 in Part II. Six domains consist of items for physical mobility (8 items), pain (8 items), social isolation (5 items), emotional reactions (9 items), energy (3 items), and sleep (5 items). This tool, which is more frequently used in Europe, is suitable for use in primary care clinical settings. Scores: Each item is weighted on the basis of findings from previous studies. The maximum sum of the items in each domain is 100. The higher the score, the greater is the health problem. Scores are presented as profiles rather than overall scores. A mean score may be calculated across all items within each domain. The overall score is the mean across all items. Accuracy: Extensive testing has indicated good reliability, test-retest/reproducibility, internal consistency, and validity for the NHP (MacDowell & Newell, 1996; Jenkinson et al, 1988). Disadvantages revolve around its limitations in measurement of function (some disabilities are not measured), inadequate index of mental distress, and inability to detect small improvements in health (Bowling, 1997). Administration Time: 5 to 10 minutes by self-administration. Contact S. McKenna for permission for use: Dr. Stephen McKenna, Galen Research, Enterprise House, Manchester Science Park, Lloyd Street North, Manchester M15 6SE, UK. This tool may be viewed online at http://www.medal.org/ch1.html. Descriptive summary from Loretz L: Primary Care Tools for Clinicians: A Compendium of Forms, Questionnaires, and Rating Scales for Everyday Practice, St. Louis, 2005, Mosby.

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Rehabilitation Functional Independence Measure (FIM™) Instrument

Copyright 1997 Uniform Data System for Medical Rehabilitations (UDSMR), a division of UB Foundation Activities, Inc. (UFBA). Reprinted with permission of UDSMR. All marks associated with FIM and UDSMR are owned by UBFA.

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Rehabilitation Functional Independence Measure (FIM): Items and Levels of Function SELF-CARE Eating. Includes use of suitable utensils to bring food to mouth, chewing, and swallowing, once meal is appropriately prepared. Grooming. Includes oral care, hair grooming, washing hands and face, and either shaving or applying makeup. Bathing. Includes bathing the body from the neck down (excluding the back), tub, shower, or sponge/bed bath. Performs safely. Dressing—Upper Body. Includes dressing above the waist as well as donning and removing prosthesis or orthosis when applicable. Dressing—Lower Body. Includes dressing from the waist down as well as donning or removing prosthesis or orthosis when applicable. Toileting. Includes maintaining perineal hygiene and adjusting clothing before and after toilet or bed pan use. Performs safely.

Check and evaluate the most usual mode of expression. If both are about equally used, check V and N. ( ) V  Vocal ( ) N  Nonvocal SOCIAL COGNITION Social Interaction. Includes skills related to getting along and participating with others in therapeutic and social situations. It represents how one deals with one’s own needs together with the needs of others. Problem Solving. Includes skills related to solving problems of daily living. This means making reasonable, safe, and timely decisions regarding financial, social, and personal affairs and initiating, sequencing, and selfcorrecting tasks and activities to solve the problems. Memory. Includes skills related to recognizing and remembering while performing daily activities in an institutional or community setting. It includes ability to store and retrieve information, particularly verbal and visual. A deficit in memory impairs learning as well as performance of tasks.

SPHINCTER CONTROL Bladder Management. Includes complete intentional control of urinary bladder and use of equipment or agents necessary for bladder control. Bowel Management. Includes complete intentional control of bowel movement and use of equipment or agents necessary for bowel control. MOBILITY Transfers: Bed, Chair, Wheelchair. Includes all aspects of transferring to and from bed, chair, and wheelchair, and coming to a standing position, if walking is the typical mode of locomotion. Transfer: Toilet. Includes getting on and off a toilet. Transfers: Tub or Shower. Includes getting into and out of a tub or shower stall. LOCOMOTION Walking or Using Wheelchair. Includes walking, once in a standing position, or using a wheelchair, once in a seated position, on a level surface. Check most frequent mode of locomotion. If both are about equal, check W and C. If initiating a rehabilitation program, check the mode for which training is intended. ( ) W  Walking ( ) C  Wheelchair Stairs. Goes up and down 12 to 14 stairs (one flight) indoors. COMMUNICATION Comprehension. Includes understanding of either auditory or visual communication (e.g., writing, sign language, gestures). Check and evaluate the most usual mode of comprehension. If both are about equally used, check A and V. ( ) A  Auditory ( ) V  Visual Expression. Includes clear vocal or nonvocal expression of language. This item includes both intelligible speech or clear expression of language using writing or a communication device.

DESCRIPTION OF THE LEVELS OF FUNCTION AND THEIR SCORES INDEPENDENT—Another person is not required for the activity (NO HELPER). 7 COMPLETE INDEPENDENCE—All of the tasks described as making up the activity are typically performed safely, without modification, assistive devices, or aids, and within a reasonable time. 6 MODIFIED INDEPENDENCE—Activity requires any one or more than one of the following: an assistive device, more than reasonable time, or there are safety (risk) considerations. DEPENDENT—Another person is required for either supervision or physical assistance in order for the activity to be performed, or it is not performed (REQUIRES HELPER). MODIFIED DEPENDENCE—The subject expends half (50%) or more of the effort. The levels of assistance required are: 5 Supervision or setup—Subject requires no more help than standby, cuing or coaxing, without physical contact. Or, helper sets up needed items or applies orthoses. 4 Minimal contact assistance—With physical contact the subject requires no more help than touching, and subject expends 75% or more of the effort. 3 Moderate assistance—Subject requires more help than touching, or expends half (50%) or more (up to 75%) of the effort. COMPLETE DEPENDENCE—The subject expends less than half (less than 50%) of the effort. Maximal or total assistance is required, or the activity is not performed. The levels of assistance required are: 2 Maximal assistance—Subject expends less than 50% of the effort, but at least 25%. 1 Total assistance—Subject expends less than 25% of the effort.

Copyright 1997 Uniform Data System for Medical Rehabilitations (UDSMR), a division of UB Foundation Activities, Inc. (UFBA). Reprinted with permission of UDSMR. All marks associated with FIM and UDSMR are owned by UBFA.

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Sexual Function Androgen Deficiency in Aging Males (ADAM) Questionnaire Name: ________________________________________________ Date: ________________________________________________

Choose the answers below that best describe how you have been feeling. Answers will help your health care provider and you to better manage your medical needs.

1. Do you have a decrease in libido (sex drive)?

Yes

No

2. Do you have a lack of energy?

Yes

No

3. Do you have a decrease in strength and/or endurance?

Yes

No

4. Have you lost height?

Yes

No

5. Have you noticed a decreased “enjoyment of life?”

Yes

No

6. Are you sad and/or grumpy?

Yes

No

7. Are your erections less strong?

Yes

No

8. Have you noticed a recent deterioration in your ability to play sports? Yes

No

9. Are you falling asleep after dinner?

Yes

No

Yes

No

10. Has there been a recent deterioration in your work performance?

Any man answering yes to #1, #7, or any three others has a high likelihood of having a low testosterone level. From Morley JE, Perry HM: Androgen deficiency in aging men, Med Clin North Am, 83(5):127–289, 1999.

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Swallowing Evaluation Burke Dysphagia Screening Test 1. 2. 3. 4. 5. 6. 7.

Has the patient had a bilateral stroke? Has the patient had a stroke involving the brainstem? Does the patient have a history of pneumonia during the acute stroke phase? Does the patient have coughing associated with feeding or during a 3-ounce water swallow test? Does the patient persistently fail to consume at least one half of meals? Is prolonged time required for feeding the patient? Is a non-oral feeding program in progress for the patient?

Yes Yes Yes Yes Yes Yes Yes

No No No No No No No

Score: A “yes” response to one or more items is a positive screen. From DePippo KL, Holas MA, Reding MJ: The Burke dysphagia screening test: validation of its use in patients with stroke, Arch Phys Med Rehabil 75(2):1284-1286, 1994. Reprinted with permission from American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation. Also described online at http://www.medal.org/ch8.html.

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Urinary Incontinence American Urological Association (AUA) Symptom Index

Last Name

First Name

Date

Mark the response correct for you and type in your score in the far right box for all SEVEN questions. 1. Incomplete emptying: Over the past month, how often have you had a sensation of not emptying your bladder completely after you finished urinating? Not at all Less than 1 Less than About half More than Almost Your Score time in 5 half the time the time half the time always 0 1 2 3 4 5 2. Frequency: Over the past month, how often have you had to urinate again less than 2 hours after you finished urinating? Not at all Less than 1 Less than About half More than Almost Your Score time in 5 half the time the time half the time always 0 1 2 3 4 5 3. Intermittency: Over the past month, how often have you found that you stopped and started again several times when you urinated? Not at all Less than 1 Less than About half More than Almost Your Score time in 5 half the time the time half the time always 0 1 2 3 4 5 4. Urgency: Over the past month, how often have you found it difficult to postpone urination? Not at all Less than 1 Less than About half More than time in 5 half the time the time half the time 0 1 2 3 4

Almost always 5

5. Weak stream: Over the past month, how often have you had a weak stream? Not at all Less than 1 Less than About half More than time in 5 half the time the time half the time 0 1 2 3 4

Almost always 5

6. Straining: Over the past month, how often have you had to push or strain to begin urination? Not at all Less than 1 Less than About half More than time in 5 half the time the time half the time 0 1 2 3 4

Almost always 5

Your Score

Your Score

Your Score

7. Nocturia: Over the past month or so, how many times did you get up to urinate from the time you went to bed until the time you got up in the morning? None 1 time 2 times 3 times 4 times 5 or more Your Score times 0 1 2 3 4 5 Add up your scores for total AUA score  _______ Quality of Life Due to Urinary Symptoms: If you were to spend the rest of your life with your urinary condition just the way it is now, how would you feel about that? (Bold, Highlight, or Underline) Delighted

Pleased

Mostly satisfied

Mixed

Mostly dissatisfied

Unhappy

Terrible

Scores: Items are scored as follows: 0 points  not at all, 1 point  less than 1 in 5 times (20%), 2 points  less than half the time (50%), 3 points  about half the time (50%), 4 points  more than half the time (50%), 5 points  almost always (80%). Scores for the seven items are summed for a total score. Suggested interpretation for total scores is as follows:  7  mild benign prostatic hyperplasia (BPH) symptoms; 8 to 19  moderate BPH symptoms; 20  severe BPH symptoms. The American Urological Association (AUA) Symptom Index is widely available online for downloading and can be found at http://www.prostate-cancer.org/tools/forms/aua_ symptom_form.html.

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Urinary Incontinence Incontinence Impact Questionnaire (IIQ-7) Name: ________________________________________________ Date: ________________________________________________

Has urine leakage affected your:

(“X” one for each question) Not at all

Slightly

Moderately

Greatly

1. Ability to do household chores (cooking, housecleaning, laundry)?

□

□

□

□

2. Physical recreation such as walking, swimming, or other exercise?

□

□

□

□

3. Entertainment activities (movies, concerts, etc.)?

□

□

□

□

4. Ability to travel by car or bus more than 30 minutes from home?

□

□

□

□

5. Participation in social activities outside your house?

□

□

□

□

□ □

□ □

□ □

□ □

6. Emotional health (nervousness, depression, etc.)? 7. Feeling frustrated?

Urogenital Distress Inventory (UDI-6) Do you experience, and if so, how much are you bothered by:

(“X” one for each question) Not at all

Slightly

Moderately

Greatly

1. Frequent urination?

□

□

□

□

2. Urine leakage related to the feeling of urgency?

□

□

□

□

5. Difficulty emptying your bladder?

□ □ □

□ □ □

□ □ □

□ □ □

6. Pain or discomfort in the lower abdominal or genital area?

□

□

□

□

3. Urine leakage related to physical activity, coughing, or sneezing? 4. Small amounts of urine leakage drops?

From Shumaker SA, Wyman JF, Uebersax JS, et al: Health-related quality-of-life measures for women with urinary incontinence, Qual Life Res 3(5);291–306, 1994.

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Vision

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Index A -fetoprotein, 46, 176 -1 adrenergic blockers, 104 -1 fetoprotein, 425 A-fib. See Atrial fibrillation AAA. See Abdominal aortic aneurysm Abciximab, 14 Abdominal aortic aneurysm definition of, 11 epidemiology and demographics of, 11 etiology of, 11 physical findings and clinical presentation of, 11 treatment of, 11 Abdominal aortic rupture, 11 Abdominal cancer, 207 Abdominal distention and ascites, 173 mechanical obstruction, 239 nonmechanical obstruction, 239 Abdominal film, for abdominal pain or evidence of obstruction, 170 Abdominal pain, 32 diffuse, 239 epigastric, 239 left lower quadrant, 239 left upper quadrant, 239 periumbilical, 239 right lower quadrant, 239 right upper quadrant, 239–240 suprapubic, 240 Abdominal trauma, 261 Abnormal chromosomal karyotype, 127 Abnormal involuntary movement scale (AIMS), 525 Abuse abandonment, 3 alcohol, 45 elder clinical presentation of, 3 definition of, 3 diagnosis of, 3 epidemiology and demographics of, 3 treatment of, 3 financial, 3 geriatric, 277 obligation, 3 phenacetin, 33 physical, 3 psychologic, 3 self-neglect, 3 sexual, 3 ACE inhibitors advantages and limitations of, 104 long-term vasodilator therapy with, 17 Acetaminophen, 218 Acetazolamide, carbonic anhydrase inhibitors, 414 Acetylcholine receptor (AChR) antibody, 411 Aches and pains, diffuse, differential diagnosis of, 240 Achilles tendinitis, 208 Acid-base reference values, 411t Acid phosphatase, 411t

Page numbers followed by b indicate boxes; f, figures; t, tables.

Acidosis metabolic, 278f–279f with increased AG acidosis, 240 with normal AG, 240 respiratory, 240 Acquired neuropathies, 264 Activated partial thromboplastin time, 434 Activities of daily living (ADL), 471, 487 Actonel (Risedronate), 128 Acute aspiration, on acidic gastric contents, 161 Acute asthma, home management of, 289f Acute bacterial prostatitis, 168 Acute brain failure. See Delirium Acute cauda equina syndrome, 194 Acute change in mental status. See Delirium Acute confusion. See Delirium Acute conjunctivitis. See Conjunctivitis Acute coronary syndrome, 12 Acute diarrhea, 306f Acute external hemorrhoids, 96 Acute glaucoma. See Primary closed-angle glaucoma Acute low back pain, management of, 290f Acute polyneuropathy. See Guillain-Barré syndrome Acute psychotic illness, 52 Acute pulmonary edema, 130 Acute red eye, 376f Acute renal failure causes of, 178, 377f and sepsis, 180 Acute scrotum, 240 Acute tubular necrosis (ATN), 178 Acute viral hepatitis, serologic tests for diagnosis of, 327f Acyclovir (Zovirax), 97 Addison’s disease, 10, 249, 251, 273 Adenocarcinoma of vulva. See Vulvar cancer Adenocarcinomas, from columnar epithelium, 72 Adenomatous polyposis coli (APC), 47–48 Adenovirus, 112 ADLs and IADLs, 75 Adrenal masses bilateral, 240–241 unilateral, 240 Adriamycin, 33 Adult immunization schedule (United States), recommended, 449t with medical conditions, 451t Advance directive documents, 469 Adynamic ileus, 241 Aeromonas hydrophila, 41 Aerosol induction, with hypertonic saline solution, 162 AF. See Atrial fibrillation AFB stain-negative sputum, 220 Age-adjusted per capita cost (AAPCC), 484 Age-related eye diseases study, on antioxidants, 231 Age-related macular degeneration (ARMD), 125, 230t Age-related senile cataracts. See Cataracts Aging cognitive changes in normal, 56 normal and abnormal changes in vision, 229 AI. See Aortic regurgitation Akinesia/bradykinesia, 152

AL amyloidosis with melphalan, treatment of, 7 AL (primary amyloidosis), 7 Alagille’s syndrome, 248 Alanine aminotransferase, 411 as liver transamines, 4 Albumin, serum, 411 Albuterol, ipratropium, and fluticasone, for nebulizer, 22 Albuterol, long-acting oral 2-agonists, 23 Alcohol abuse. See Alcoholism Alcohol and benzodiazepines, 15 Alcohol consumption, 81 Alcoholism, 280f definition of, 4 diagnosis of, 4 epidemiology and demographics of, 4 etiology of, 4 pharmacotherapies for, 5 physical findings and clinical presentation of, 4 treatment of, 4 Aldolase, serum, 412 Aldosterone receptors, blockade of, 91 Alendronate, 148 Alfuzosin, 31 Alkaline phosphatase, 412 Alkalosis metabolic, 281f chloride-resistant, 241 chloride-responsive, 241 respiratory, 241 Allergic and nonallergic rhinosinusitis, 116 Allergic rhinosinusitis. See Rhinosinusitis, allergic Allergies, 85 Allopurinol, xanthine oxidase inhibitor, 86 Alprazolam, 74 ALS. See Amyotrophic lateral sclerosis ALS-Parkinsonism-dementia complex, 8 ALT. See Alanine aminotransferase Alteration of diurnal rhythms, 110 Alveolar-arteriolar oxygen gradient, 171 Alveolar consolidation, 241 Alzheimer’s disease, 7, 113, 262, 477 cause of dementia, 54 cognitive features of, 54 core features of, 54 genetics and family history of, 54 key neuroimaging features of, 54 neurologic features of, 54 pathology of, 54 prevalence of, 54 Amantadine (Symmetrel), 153 Amaurosis fugax, 230t American Academy of Sleep Disorders, 192 American College of Cardiology/American Heart Association on heart failure, 90 American College of Rheumatology’s primary condition, for fibromyalgia, 80 American Diabetes Association (ADA), 62 criteria, 63 exchange diet of, 63 American Psychiatric Association’s diagnostic criteria for alcohol withdrawal, 4 American Rheumatism Association, revised diagnostic criteria for CPPD, 169 American Tinnitus Association, 213 American Urological Association (AUA), 166 guideline recommendations, 34b

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Index American Urological Association—cont’d symptom index, 554 for ureteral stones, 225 Amiodarone, as drug toxicity, 163 Ammonia, serum, 412 Ammonium chloride, exogenous acids, 414 Amnesia, 241 Amoxapine, drug for depression, 204 Amphetamine, 15 Amylase, serum, 412 Amyloid, 7 Amyloidosis definition of, 7 diagnosis of, 7 epidemiology and demographics of, 7 etiology of, 7 physical findings and presentation of, 7 as a systemic disorder, 108 treatment of, 7 Amyotrophic lateral sclerosis definition of, 8 diagnosis of, 8 epidemiology and demographics of, 8 etiology of, 8 physical findings and clinical presentation of, 8 treatment of, 8 ANA. See Antinuclear antibody Anal incontinence mass effect, 241 medical, 241 neurologic, 241 traumatic, 241 Anaphylaxis cardiovascular, 241 CNS, 241 endocrine, 241 psychiatric, 241 pulmonary, 241 Anarthritic rheumatoid syndrome, 164 ANCA. See Antineutrophil cytoplasmic antibody Androgen deficiency in aging males (ADAM), 552 Anemia, 47, 59 algorithm for diagnosis of, 282f drug-induced, 242 and erythrocytosis, 176 with GI bleeding, 154 iron deficiency definition of, 9 differential diagnosis of, 9 epidemiology and demographics of, 9 etiology of, 9 physical findings and clinical presentation of, 9 treatment of, 9 low reticulocyte count macrocytic, 242 microcytic, 242 normocytic, 242 and macrocytosis, absence of, 10 major complaints of, 9 megaloblastic cobalamin (CBL) deficiency, 242–243 folate deficiency, 242 microcytic, 66 pernicious, 10 definition of, 10 diagnosis of, 10 epidemiology and demographics of, 10 etiology of, 10 physical findings and clinical presentation of, 10 treatment of, 10 sideroblastic, 9

Anemia—cont’d and thrombocytopenia, 117 with variable MCV, 134 Anergy antigen testing, 220 Aneurysms, thoracic aorta, 243 Angina, grading by Canadian Cardiovascular Society Classification System, 12 modifiable risk factors for, 12 New York Heart Association Functional Classification of, 12 noncardiac pain mimicking, 12 uncontrollable risk factors for, 12 unstable, 135 Angina pectoris, 12, 154 definition of, 12 diagnosis of, 12 epidemiology and demographics of, 12 physical findings and clinical presentation of, 12 treatment of, 13 Angiography, gold standard for visualizing arterial anatomy, 156 Angioplasty and coronary stents, 14 in iliac or femoropopliteal artery, 157 Angiotensin II receptor blockers (ARB), advantages and limitations of, 104 Ankle-brachial index (ABI), 156 Ankylosing spondylitis, 194 Ann Arbor classification, 123 Annular lesion (Tinea corporis), 211f Anorexia, evaluation of, 286f Anserine bursitis. See Bursitis, anserine Anserine tendinitis. See Bursitis, anserine Antacids, for relief of mild symptoms, 82 Anterior chamber paracentesis, 85 Anterior ischemic optic neuropathy (AION), 83, 230t Anterior thalamus, single subcortical strategic infarct, 55 Anti-DNA, 412 Anti-RNP antibody, 412 Anti-SM (Anti-Smith) antibody. See Extractable nuclear antigen Antiandrogens, 167 Antibiotic-induced colitis. See Pseudomembranous colitis Antibiotics, administration of, 170 Antibodies, serologic testing for, 154 Antibody-coated bacteria with pyelonephritis, 223 Anticardiolipin antibody, 412 hematologic disorders, 208 Anticholinergic drugs, 111, 153 Anticoagulant. See Circulating anticoagulant Antigens, guidelines for spacing of live and inactivated, 456t Antihistamines, as anti-cholinergic drugs, 31 Antihypertensives, 59 Antiischemic agents, classes of, 13 Antimitochondrial antibody, 412 Antineutrophil cytoplasmic antibody, 412 Antinuclear antibody, 412 in autoimmune hepatitis, 46 subtypes, disease associated with, 413t 5 test patterns, fluorescent, 413f with anti-SS A/anti-SS B, 191 Antiplatelet therapy, on stroke, 200 Antipsychotics, sedating, for severe mood or psychotic disorders, 114 Antistreptolysin O titer, 414 Antithrombin III deficiency, hematologic disorders, 208 Antithyroid drugs (thionamides), 107

Anxiety definition of, 15 diagnosis of, 15 epidemiology and demographics of, 15 etiology of, 15 physical findings and clinical presentation of, 15 treatment of, 15 Anxiety neurosis. See Anxiety Aortic dissection, 16 classification of, 16, 16f definition of, 16 diagnosis of, 16 epidemiology and demographics of, 16 etiology of, 16 physical findings and clinical presentation of, 16 treatment of, 16 Aortic insufficiency. See Aortic regurgitation Aortic regurgitation, 17, 246 definition of, 17 diagnosis of, 17 epidemiology and demographics of, 17 etiology of, 17 physical findings and clinical presentation of, 17 treatment of, 17 Aortic sclerosis, 18 Aortic stenosis, 246 balloon aortic valvotomy for adult acquired, 18 definition of, 18 diagnosis of, 18 epidemiology and demographics of, 18 etiology of, 18 physical findings and clinical presentation of, 18 treatment of, 18 Aortic valvular stenosis. See Aortic stenosis Apathetic hyperthyroidism, 107 Apolipoprotein and plasma viscosity, 156 AR. See Aortic regurgitation ARF, 178 Arnold-Chiari malformation, 263 Arrhythmias/asystole, 202 Arsenic, heavy metal poisoning, 88 Arterial anatomy, angiography on, 11 Arterial blood gases, 414 Arterial embolization with atrial fibrillation, 190 Arterial occlusion, 243 Arteritis, inflammatory, 11 Artery anterior cerebral, 198t anterior inferior and superior cerebellar, 198t anterior spinal, 198t internal carotid, 198t middle cerebral, 198t posterior cerebral, 198t posterior inferior cerebellar, 198t posterior spinal, 198t vertebral or basilar, 198t Arthralgia, diagnostic approach for, 287f Arthritis, 77 monoarticular and oligoarticular, 243 polyarticular, 243–244 rheumatoid definition of, 19 diagnosis of, 19 epidemiology and demographics of, 19 etiology of, 19 physical findings and clinical presentation of, 19 treatment of, 19 Arthrocentesis fluid, 414

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Index Arthrosis. See Osteoarthritis AS. See Aortic stenosis Ascending aorta DeBakey type I, II and III, 16 Stanford type A, 16 Ascending paralysis. See Guillain-Barré syndrome Ascites, 244 evaluation of, 288f Aseptic necrosis definition of, 21 diagnosis of, 21 epidemiology and demographics of, 21 etiology of, 21 of hips, 21f physical findings and clinical presentation of, 21 treatment of, 21 Asherman’s syndrome, 127 ASLO titer. See Antistreptolysin O titer Aspartate aminotransferase, 414 Aspiration pneumonia. See Pneumonia, aspiration Aspiration pneumonitis, 187 Aspiration/steroid injection, 169 Aspiration, with gram stain and C&S, 37 Aspirin, 13 for acute MI, 135 with cardiovascular disease, 156 chemoprophylaxis with, 48 and dipyridamole extended-release capsules, 214 Assisted living, 471, 471t AST, as liver transamines, 4 Asteatotic eczema, 196 Asthma, 171 acute, 298f American Thoracic Society definition on, 22 definition of, 22 diagnosis of, 22 drug-induced, 22 epidemiology and demographics of, 22 etiology of, 22 exercise-induced, 22 extrinsic, 22 intrinsic, 22 physical findings and clinical presentation of, 22 quick relief of, 23 treatment of, 22 Asymptomatic hematuria, algorithm for evaluation of, 326f At-risk drinking, definition of, 4 Ataxia, 244 Athlete’s foot. See Tinea pedis Atopic dermatitis, 186, 196 Atrial fibrillation, 129 calcium channel blocker, 18 definition of, 24 diagnosis of, 24 digoxin during, 24 epidemiology and demographics of, 24 esmolol, metoprolol, atenolol used during, 24 etiology of, 24 physical findings and clinical presentation of, 24 procainamide during, 24 treatment of, 24 Atrophic gastritis, endoscopy and biopsy for, 10 Atrophic vaginitis. See Vulvovaginitis, estrogen-deficient AUDIT, screening test for alcoholism, 4 Austin-Flint murmur, 17 Avascular necrosis. See Aseptic necrosis

Axilla and supra-clavicular areas, nodal enlargement in, 35 Azelastine with loratadine/fexofenadine, 184 Azidothymidine (AZT), 242 Azithromycin/clarithromycin, 162

B -2 microglobulin levels, 123 -adrenergic agonists, 256 -adrenergic blockers, antiangial effect of, 13 -adrenergic blocking agents, with evolving acute MI, 136 -adrenergic bronchodilators, 74 -agonist albuterol (Combivent), 44 -blockers, 24, 85, 91 advantages and limitations of, 104 to reduce myocardial oxygen consumption, 136 and verapamil, 92 -human chorionic gonadotropin, 176 B-type natriuretic peptide (BNP), 93, 415 B12 deficiency, as metabolic disorder, 56 Babinski’s sign, 261 Back pain, 244 management of, 290f Bacterial pneumonia definition of, 162 diagnosis of, 162 epidemiology and demographics of, 162 etiology of, 162 physical findings and clinical presentation of, 162 secondary, 112 treatment of, 162 Bacterial prostatitis acute, 168 chronic, 168 Bacterial-viral pneumonia, mixed, 112 Baker’s cyst as gastrocnemius-semimembranosus bursa, 37 ruptured, 208 Balloon valvuloplasty, for short-term reduction in severity of aortic stenosis, 18 Barium enema, 66 Barium swallow, 73t Barrett’s esophagus definition of, 26 diagnosis of, 26 epidemiology and demographics of, 26 etiology of, 26 with mucosal biopsy, 82 physical findings and clinical presentation of, 26 treatment of, 26 Bartholin gland carcinoma. See Vulvar cancer Bartter’s syndrome, 256 Basal cell carcinoma, 28f definition of, 28 diagnosis of, 28 epidemiology and demographics of, 28 etiology of, 28 physical findings and clinical presentation of, 28 treatment of, 28 Basal cell carcinoma of vulva. See Vulvar cancer Bassen-Kornzweig disease, 264 Battered elder syndrome. See Abuse, elder BCC. See Basal cell carcinoma Bedsores. See Wounds Behçet’s syndrome, 244, 247, 250, 272

Bell’s palsy definition of, 29 diagnosis of, 29 epidemiology and demographics of, 29 etiology of, 29 physical findings and clinical presentation of, 29 treatment of, 29 Bell’s phenomenon, 29 Bence Jones protein, 132 Benign prostatic hyperplasia, 30–31 as asymptomatic, 30 definition of, 30 epidemiology and demographics of, 30 etiology of, 30 physical findings and clinical presentation of, 30 prostate enlargement caused by, 31 treatment of, 31 Benign prostatic hypertrophy, 371f Benzodiazepine receptor agonists, 114 Benzodiazepines, 183, 213 as second line therapy, 58 and vitamin E, 204 Berg balance scale, 526–527 Berger’s disease, 253 Bethesda guidelines, for microsatellite instability testing, 48 Bevacizumab, 177 Bicalutamide, as antiandrogens, 167 Biliary liver disease, 115 Bilirubin direct, 415 indirect, 415 total, 415 Bismuth compound, 155 Bisphosphonates, 148 “Black” tobacco, 32 Bladder accidents. See Urinary incontinence Bladder cancer, 32–34 definition of, 32 diagnosis of, 33 epidemiology and demographics of, 32 etiology of, 33 treatment of, 33 Bleeding evaluation of diverticular, 66 gastrointestinal, 291f lower GI, 244–245 upper GI, 245 Blepharospasm. See Dystonia Blessed dementia scale and informationmemory concentration test, 507 Blindness. See Vision loss “Blond” tobacco, 32 Blood, lymphocytes abnormality in peripheral, 432t Blood pressure and heart rate, 202 Blood sugar and CBC, 85 Blood urea nitrogen, 441 Blue bloaters (chronic bronchitis), 43 Blue rubber bleb nevus syndrome, 255 Body mass index, 532 definition of, 139 Body ringworm. See Tinea corporis Bone fracture, 319f Bone infection. See Osteomyelitis Bone lesions, preferential site of origin diaphysis, 245 epiphysis, 245 metadiaphyseal, 245 metaphysis, 245 Bone metastasis, palliative radiation therapy for, 73 Bone mineral density (BMD), 146 age and T score for, 146f Bone neoplasms, 132

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Index Bone scan, to evaluate bone metastasis, 166 “Booster phenomenon”, 220 Bortezomib (Velcade), 133 Botulinum toxin, for treatment of synkinesis and hemifacial spasm, 29 Bowen’s disease, 272 BPH. See Benign prostatic hyperplasia Brachytherapy and teletherapy, 70 Bradycardia general approach to, 292f sinus, differential diagnosis of, 245 Bradycardia-tachycardia syndrome. 190f See also Sick sinus syndrome Bradykinesia/akinesia, 152 Brain imaging, 52 Brain tumor, 187 Bran, 63 “Brawny” edema, 196 Breast cancer, 35 definition of, 35 diagnosis of, 35 epidemiology and demographics of, 35 etiology of, 35 physical findings and clinical presentation of, 35 screening and evaluation of, 295f, 296 treatment of, 35 Breast mass, differential diagnosis of, 245 Breath odor, differential diagnosis of, 245 Bromocriptine (Parlodel), 153 Bronchial asthma. See Asthma Bronchospasm. See Asthma Budd-Chiari syndrome, 46, 244 Bullous diseases, differential diagnosis of, 246 BUN and creatinine, 31 elevated, 178 to rule out renal disease, 103 Bupropion (Wellbutrin), 140 Burke dysphagia screening test, 553 Burkitt’s lymphoma, 254 Burow’s solution, 212 Bursa around knee, 37f injection, 38 swollen prepatellar, 37f Bursitis definition of, 37 diagnosis of, 37 etiology of, 37 physical findings and clinical presentation of, 37 treatment of, 37 Bursitis, anserine definition of, 38 diagnosis of, 38 epidemiology and demographics of, 38 etiology of, 38 physical findings and clinical presentation of, 38 treatment of, 38 Butenafine Hcl (Mentax), 212

C C. difficile-associated diarrhea, 170 C-peptide, 421 C-reactive protein, 83, 421 as acute phase reactants, 19 elevated levels of, 156 CABG surgery, for left main coronary disease, 14 CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), 54 Caffeine, 74

CAGE questionnaire, 4 Calcitonin nasal spray, 148 Calcium channel blockers, 13, 174 advantages and limitations of, 104 and antiarrhythmic agents, 90 Calcium chloride, exogenous acids, 181, 414 Calcium oxalate/calcium phosphate stones, for urolithiasis, 224 Calcium pyrophosphate dehydrate (CPDD), 169 Calcium, serum, 415 Calcium stones, radioopaque stones, 225 Cancer bladder. See Bladder cancer breast. See Breast cancer colorectal (colon cancer). See Colorectal cancer endometrial. See Endometrial cancer gastric. See Gastric cancer head and neck. See Laryngeal cancer leukemia. See Chronic lymphocytic leukemia lung. See Lung neoplasms, primary ovarian, See Ovarian cancer pancreatic, See Pancreatic cancer prostate, See Prostate cancer skin. See Squamous cell carcinoma testicular, 392 vulvar, See Vulvar cancer CAP (cyclophosphamide, adriamycin, prednisone), 117 Capsaicin cream (Zostrix), 97 Carbamazepine, 188, 213, 216 Carbonmonoxide, as toxins, 152 Carcinoembryonic antigen (CEA), 47, 416 Carcinoid syndrome, 202, 250 Carcinoid tumor, 121 Carcinoma-in-situ (CIS), 32 Carcinoma, non-small cell, 122 Cardiac arrest, nontraumatic, differential diagnosis of, 246 Cardiac catheterization, 18 Cardiac enlargement, differential diagnosis of, 246 Cardiac failure. See Congestive heart failure Cardiac isoenzymes, 13, 202 Cardiac murmurs, differential diagnosis of, 246–247 Cardiac troponin I and T, 13 Cardio-CRP, 13 Carotene, serum, 416 Carotid endarterectomy (CEA), 199 for carotid territory TIA, 214 Carotid sinus pressure, 202 Carpal compression test, 39 Carpal tunnel syndrome, 7, 39, 158, 217 definition of, 39 diagnosis of, 39 epidemiology and demographics of, 39 etiology of, 39 physical findings and clinical presentation of, 39 treatment of, 39 Cat-scratch disease, 261 Cataracts, 40, 230t central location of posterior subcapsular, 40f definition of, 40 diagnosis of, 40 epidemiology and demographics of, 40 etiology of, 40 and glaucoma, 62 physical findings and clinical presentation of, 40 treatment of, 40 Cauda equina syndrome, 119 acute, 194

CBC. See Complete blood count CEA. See Carcinoembryonic antigen Cefepime/piperacillin-tazobactam, 163 Celery, insoluble fiber, 63 Celiac sprue, 170 Cellulitis, 41 definition of, 41 diagnosis of, 41 epidemiology and demographics of, 41 etiology of, 41 physical findings and clinical presentation of, 41 treatment of, 41 Center for epidemiologic studies depression scale (CES-D), 520 Centers for Medicare & Medicaid Services (CMS), 473, 477, 484, 489 Central nervous system disorder, 418t–419t Central spinal stenosis. See Spinal stenosis Cephalosporins, in pseudomembranous colitis, 170 Cerebellar tremor, 74 Cerebral angiogram (four-vessel), 214 Cerebral ischemia, evaluation on, 298 Cerebrospinal fluid, 416 Cerebrovascular accident, 197 Cerebrovascular disease, ischemic, 247 Ceruloplasmin, serum, 420 Cervical spondylosis, algorithm for treatment of, 387f Cetirizine (Zyrtec), 226 Cevimeline, 191 Chagas’ disease, 259 Change of life. See Menopause Charcot-Marie-Tooth syndrome, 158, 216, 263 Charcot’s joints, 63 Chédiak-Higashi syndrome, 255 Chemotherapy and tamoxifen, 70 Chest pain nonpleuritic, 247 pleuritic, 247 Chest x-ray, 121 calcification on, 246 cardiomegaly on, 297f cavitary lesion on, 247 to rule out infectious process, 100 CHF. See Congestive heart failure Chlorambucil chemotherapy with prednisone, 117 without prednisone, 117 Cholangiocarcinoma, 151 Cholelithiasis, 42 definition of, 42 diagnosis of, 42 epidemiology and demographics of, 42 etiology of, 42 physical findings and clinical presentation of, 42 Cholelithiasis syndrome, 154 Cholestasis, pleuritic, 248 Cholesterol, high-density lipoprotein. See High-density lipoprotein cholesterol Cholesterol, low-density lipoprotein. See Low-density lipoprotein cholesterol Cholestyramine, to control diarrhea, 170 Chondrocalcinosis. See Calcium pyrophosphate dehydrate Chronic anticoagulation with warfarin, 174 Chronic anxiety. See Anxiety Chronic atrophic gastritis with intestinal metaplasia, 81 Chronic bacterial prostatitis, 168 Chronic bronchitis. See Chronic obstructive pulmonary disease Chronic conjunctivitis. See Conjunctivitis Chronic cough, 301f Chronic diarrhea, 307f

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Index Chronic diseases anemia of, 9 evaluation of, 75 Chronic fatigue syndrome, 240 Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 158 Chronic inflammatory diseases, 7 Chronic inflammatory process, with collagen vascular disease or sarcoidosis, 231 Chronic lymphocytic leukemia, 117 diagnosis of, 117 epidemiology and demographics of, 117 etiology of, 117 physical findings and clinical presentation of, 117 treatment of, 117 Chronic obstructive pulmonary disease, 43–44, 173, 299 acute exacerbation of, 43 definition of, 43 diagnosis of, 43 epidemiology and demographics of, 43 etiology of, 43 physical findings and clinical presentation of, 43 treatment of, 43 Chronic open-angle glaucoma definition of, 84 diagnosis of, 84 epidemiology and demographics of, 84 etiology of, 84 Chronic pancreatitis, 115 Chronic renal failure (CRF), 181 Chronic simple glaucoma. See Chronic openangle glaucoma Chronic subdural hematomas, 201 Chronic venous stasis ulcers, 196 Churg-Strauss syndrome, 254 Ciclopirox, 142, 212 Cilostazol (Pletal), 157 Cimetidine, 226 Cinacalcet (Sensipar), 102 Ciprofloxacin (antibiotic drops), 49 Circulating anticoagulant, 420 Cirrhosis, 45, 178 definition of, 45 diagnosis of, 45 epidemiology and demographics of, 45 etiology of, 45 physical findings and clinical presentation of, 45 treatment of, 46 Cisapride and terfenadine, 142 Cisplatin and etoposide, 122 “Classic flu”, 112 Clear cell carcinoma of the kidney. See Renal cell adenocarcinoma Climacteric ovarian failure. See Menopause Clinical Institute Withdrawal AssessmentAlcohol (CIWA-A) scale, 4–5 CLL. See Chronic lymphocytic leukemia Clock drawing test, 508 Clopidogrel, an antiplatelet agents, 13, 136 Clopiogrel, with PAD, 156 Clostridium difficile colitis. See Pseudomembranous colitis Clostridium difficle toxic assay (stool), 420 Clotrimazole cream (Mycelex), 211 Clotrimazole (Lotrimin AF), 212 Clozapine antipsychotic drugs, 204 olanzapine and amisulpride, 204 and quetiapine, 204 Clubbing, differential diagnosis of, 248 CMV (cisplatin, methotrexate vinblastine) therapy, 33 CNS infection, 187

CO2 laser revascularization, 14 Coagulation factor deficiencies, screening laboratory results in, 421t Coagulation factors, characteristics of, 420t Cobalamin laboratory test for detecting, 10 RIA measurement, radioactivity interferes with, 10 Cocaine, 15 Cockroft-Gault formula, 423b Cognitive-behavioral therapy (CBT), 60 for anxiety and insomnia-perpetuating behaviors, 113 Collagen vascular disease. See Cataracts Colon left side of, 47 right side of, 47 Colonoscopy with biopsy, 47 Colorectal cancer, 47–48 definition of, 47 diagnosis of, 47 Dukes’ and UICC classification for, 47 epidemiology and demographics of, 47 etiology of, 47 treatment of, 47–48 Colpocleisis, 228 Common duct stricture, 151 Comorbid heart failure, 44 Complement deficiency states, 422t Complete blood count, 187, 223, 421 for detecting anemia, 192 and ESR, 38 with platelets, 214 Condylomata acuminata, 96 Confusion assessment method (CAM), 515 for detecting delirium, 52 for intensive care unit, 517 Congenital cataracts. See Cataracts Congenital heart disease, 173 Congestive heart failure, 59, 93, 178, 208 and arrhythmias, 192 definition of, 90 diagnosis of, 90 epidemiology and demographics of, 90 etiology of biventricular failure, 90 left ventricular failure, 90 right ventricular failure, 90 hepatic congestion, 45 physical findings and clinical presentation of, 90 with preserved LV function, 93 systemic disorders, 108 treatment of, 91–92 Conjugated bilirubin. See Bilirubin, direct Conjunctivitis, 49 definition of, 49 diagnosis of, 49–50 epidemiology and demographics of, 49 etiology of, 49 treatment of, 49–50 viral, 49f Constipation, 51, 300f definition of, 50 differential diagnosis of, 248 epidemiology and demographics of, 50 etiology of, 50 pharmacologic management of, 51t Contact dermatitis, 186, 196 Contact lens, wearing with irritation, 85 Continuous hormone replacement therapy, 128 Continuous positive airway pressure (CPAP), for sleep apnea, 192 Continuous subcutaneous insulin infusion (CSII), 64

Contrast venography, for evaluation of DVT, 208 Convulsive disorders. See Seizure disorders Coombs direct, 421 indirect, 421 COPD. See Chronic obstructive pulmonary disease Copper, serum, 421 Cornell scale for depression in dementia (CSDD), 521–522 Coronary angiography for identification of coronary artery disease, 13 indications for, in perioperative evaluation before noncardiac surgery, 539t Coronary artery bypass grafting (CABG), 196 Coronary artery disease, preoperative evaluation of, 369f Coronary occlusion. See Myocardial infarction Coronary stents, development of, 14 Coronary syndrome, acute, 12 Coronary thrombosis. See Myocardial infarction Corrigan’s pulse, 17 Corticosteroid, low-dose inhaled, 22 Cortisol, plasma, 421 Cough chronic, 301f differential diagnosis of, 248 CPAP therapy, 192 CPDD. See Calcium pyrophosphate dehydrate CPT codes, 480t for nursing facility services, 488t Cranial arteritis. See Giant cell arteritis “Crazy legs”. See Restless legs syndrome Creatine clearance, calculation of, 423b Creatine kinase elevation, evaluation of, 423f Creatine kinase isoenzymes, 423 Creatine kinase MB isoenzyme, 135 Creatinine, serum, 423 Creatinine, urine microalbumin, lipids, monitoring, 63 CRF. See Chronic renal failure “Crocodile tongue”, 191f Crohn’s disease, 250, 272 Cromolyn (Intal), 22 Cryoglobulins, serum, 424 Cryosurgery with liquid nitrogen, 28 Cryptococcus neoformans (fungi), 41 Cryptogenic seizures, 187 CSF. See Cerebrospinal fluid CT angiography for diagnosing PE, 172 vs. standard pulmonary angiography, 172 CT scan of adenocarcinoma, 151f to assess extent of prostate cancer, 166 for detecting mass lesions, 46 to diagnose acute diverticulitis, 66 of head, 100 for preoperative aneurysm imaging and estimating size, 11 Cushing’s syndrome, 63, 68, 103, 145, 249, 256, 269, 273 and chronic steroid therapy, 103 CVA. See Cerebrovascular accident CXR, on occult pneumonia, 52 Cyanosis, 302f differential diagnosis of, 248–249 Cystine exogenous acids, 414 stones, 225 Cystitis, defense mechanisms against, 222

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Index D D-dimer, 424 measurement of, 209 D-xylose absorption, 424 Daily living, index of independence in activities of, 537 Darifenacin, 111 De Musset’s sign, 17 De Quervain’s tenosynovitis, 217 Decubitus ulcers. See Wounds Deep venous thrombophlebitis. See Thrombosis, deep vein Degenerative joint disease. See Osteoarthritis Dehydration, during constipation, 50 Delirium, 52–53 definition of, 52 diagnosis of, 52 differential diagnosis of deficiency states, 249 endocrine disorders, 249 inflammatory disorders, 249 metabolic disorders, 249 neurologic disorders, 249 organ failure, 249 pharmacologic agents, 249 epidemiology and demographics of, 52 etiology of, 52 geriatric patient, 303 as multifactorial syndrome, 52 physical findings and clinical presentation of, 52 treatment, 52 types of medications for, 53 Delirium tremens with alcohol withdrawal, 52 Dementia, 52, 54 vs. amnesia, 57 vs. aphasia, 57 definition of, 54 vs. delirium, 57 vs. depression, 57 diagnosis of, 57 for diagnosis of probable DLB, 56 epidemiology and demographics of, 54 etiology of, 54 evaluation of, 55f management of, 304f miscellaneous causes of, 56 physical findings and clinical presentation of, 54 potentially reversible causes of, 56 screening tests for diagnosis of, 304t treatment of, 58 Dementia with lewy bodies (DLB) cognitive features of, 56 features of, 56 genetics and family history of, 56 neuroimaging features of, 56 neurologic features of, 56 pathology of, 56 prevalence of, 56 Depression, 52 treatment of, 60, 305f Depressive disorder, major, 59–60 definition of, 59 diagnosis of, 59 epidemiology and demographics of, 59 etiology of, 59 physical findings and clinical presentation of, 59 treatment of, 60 Dermal ulcers. See Wounds Dermatitis herpetiformis, 186 Desloratadine, 184 Dexamethasone (VAD), 132

Diabetes Control and Complications Trial (DCCT), 65 Diabetes hepatitis. See Nonalcoholic fatty liver disease Diabetes insipidus, 63 Diabetes mellitus comparison of common types of, 62t definition of, 62 diagnosis of, 63 differential diagnosis of, 63 epidemiology and demographics of, 62 etiology of, 63 physical findings and clinical presentation of, 62 treatment of, 63 as type 1, 62 as type 2, 62 Diabetic ketoacidosis (DKA), 256 Diabetic retinopathy, 230t Diamox, 85 DIAPPERS, 110 Diarrhea acute, 306f chronic, 224, 307f with GI involvement, 7 Diastolic dysfunction, 93 Diastolic heart failure, 93 Diazepam, for prolonged seizure activity, 5 Diffuse esophageal spasm, 72 Diffusion weighted imaging (DWI), on hyperacute ischemia, 197–199 Digital rectal examination (DRE), 30, 166 Digital stenosing tenosynovitis, 217 Digoxin, 75 Dihydroergotamine, 21 Diltiazem calcium channel blockers, 181 and verapamil, 104 Diphenhydramine, 226 Diplopia, binocular, differential diagnosis of, 249 Direct fluorescent antibody (DFA), 162 Direct penile damage, 71 Directly observed therapy (DOT), 221 Discus inhaler (Advair), 23 Disease-modifying drugs, 19–20 Dissection of aorta, 171 Distal and lateral subungual onychomycosis (DLSO), 141 Distal right arm, focal dystonia of, 69f Disulfiram, medications, 88 Diuretics advantages and limitations of, 104 improve dyspnea and peripheral edema, 174 Diverticular disease (diverticulosis, diverticulitis), 66 definition of, 66 diagnosis of, 66 epidemiology and demographics of, 66 etiology of, 66 physical findings and clinical presentation of, 66 treatment of, 66 Diverticulitis, 115 cause muscle spasm, 66 WBC count in, 66 DLB. See Dementia with lewy bodies Do not attempt resuscitation (DNAR) order, 470 Do not resuscitate (DNR) order, 470 Donepezil, acetylcholinesterase inhibitors, 58 Dopamine receptor agonists, 153 Dopaminergic drugs, 183 “Double Duroziez” murmur, 17 Dowager’s hump, 145

Doxazosin -1 blockers to relax smooth muscle of bladder neck, 31 -adrenergic blocker, 168 Doxepin, a tricyclic antidepressant, 226 Driver, elderly, 472 “Drop-arm” test, 185 Drug-induced cholestasis, 151 DSM-IV-R criteria, screening instrument for alcoholism, 4 Duodenal ulcer, maintenance therapy in, 154–155 DuoDerm, 196 Dupuytren’s contracture, 67, 67f, 217 definition of, 67 differential diagnosis of, 67 epidemiology and demographics of, 67 physical findings and clinical presentation of, 67 treatment of, 67 DVT. See Thrombosis, deep vein Dysfibrinogenemias, hematologic disorders, 208 Dysmyelopoietic syndrome. See Myelodysplastic syndrome Dyspareunia, 118, 233 differential diagnosis of, 249 Dyspepsia, algorithm for evaluation of, 308f Dysphagia causes of esophageal, 68 causes of oropharyngeal, 68 definition of, 68 diagnosis of, 68 differential diagnosis of, 249–250, 309f epidemiology and demographics of, 68 etiology of, 68 physical findings and clinical presentation of, 68 Dyspnea acute, 310–311 chronic, 312f differential diagnosis of, 250 on eyelids, 69 evaluation of patient with, 310f Dystonia, 69 definition of, 69 differential diagnosis of, 69 epidemiology and demographics of, 69 etiology of, 69 on eyelids, 69 physical findings and clinical presentation of, 69 treatment of, 69 Dysuria, differential diagnosis of, 250

E Eaton-Lambert syndrome, 121 Echocardiography for detecting related heart disease, 192 to evaluate left atrial size, 24 to show T-wave inversion or ST-segment, 12 Econazole (Spectazole), 211 Eczema, 212 Edema evaluation of generalized, 313f evaluation of regional, 314f generalized, 250 leg, unilateral with pain, 250 without pain, 250 of lower extremities, 250 EEG, 187 Ehlers-Danlos syndrome, 16, 131 Elder abuse. See Abuse, elder Electroconvulsive therapy (ECT), 60, 470

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Index Electrolytes, 178 Electromyography (EMG), 194 Electrophoresis, hemoglobin. See Hemoglobin electrophoresis Electrophoresis, protein. See Protein electrophoresis Elevated BUN, 178 Elevated serum creatinine, 178 Eloxatin, an inhibitor of DNA synthesis, 48 Emergency medical service (EMS) teams, 470 Emphysema. See Chronic obstructive pulmonary disease ENA-complex. See Extractable nuclear antigen End-stage renal disease. See Chronic renal failure Endocarditis, cardiac conditions with, 460b Endocarditis prophylaxis dental procedures and, 460b procedures and, 461b EndoCinch procedure, 82 Endolymphatic hydrops. See Meniere’s disease Endometrial cancer, 70 definition of, 70 diagnosis of, 70 epidemiology and demographics of, 70 etiology of, 70 physical findings and clinical presentation of, 70 treatment of, 70 Endometriosis, 66 Endometrium, carcinoma of, 70f Endomysial antibodies, 424 Endoscopic retrograde cholangiopancreatography (ERCP), 151 Endoscopy, to identify ulceration, 154 Endothelin-receptor antagonist, 174 Enoxaparin (Lovenox), 208 Entacapone (Comtan), 153 Enteric parasites, 170 Enterococcus, 144 Enzyme-linked immunoabsorbent assay (ELISA), 170 Epidermal growth factor receptor (EGFR), 48, 122 Epilepsy differential diagnosis of, 250 drug of choice in treatment of, 188t Epistaxis, differential diagnosis of, 251 Epithelial metaplasia, 27f Epithelial ovarian cancer. See Ovarian cancer Epoprostenol, or prostacyclin, continuous infusion of, 174 Epstein-Barr viral infection antibody tests in, 425t tests in, 424f Eptifibatide (Integrilin), 14 Erectile dysfunction, 71 clinical presentation of, 71 differential diagnosis of, 71 epidemiology and demographics of, 71 etiology of, 71 organic, differential diagnosis of, 251 treatment of, 71 Erysipelas. See Cellulitis Erysipelothrix rbusiopathiae, 41 Erythrocyte sedimentation rate (ESR), 164, 424 Erythropoietin, 134, 424 for anemia, 117, 181 Esophageal dysphagia. See Dysphagia Esophageal rings and webs, 72 Esophageal spasm, 82 Esophageal tumors, 72–73 definition of, 72

Esophageal tumors—cont’d diagnosis of, 72 epidemiology and demographics of, 72 etiology of, 72 physical findings and clinical presentation of, 72 radiation therapy, 73 treatment of, 72 Esophagitis, 82 Esophagus achalasia of, 72 scleroderma of, 72 ESR, 214 as acute phase reactants, 19 Essential hypertension. See Hypertension Estradiol, serum, 425 Estrogen, 425 with birth control pills, 171 replacement therapy, 189 Eszopiclone and zolpidem CR, 114 Etanercept (Enbrel), 20 Ethanol, 425 Etoposide (VP16), 33 Eucerin, 196 European Society of Cardiology/American College of Cardiology, criteria for acute evolving or recent MI, 135 Evista (Raloxifene), 128 Evoxac, 191 Exertional dyspnea, with orthopnea and PND, 130 Expression of prostatic secretions (EPS), 168 Extractable nuclear antigen, 425 Extracorporeal shock wave lithotripsy (ESWL), 42, 225 Eye pain, differential diagnosis of, 251 EZH2 protein, in prostate cancer, 167

F Fabry’s disease, 262, 264 Facial pain, differential diagnosis of, 251 Facial paralysis, differential diagnosis of, 251 Failure to thrive, 75 definition of, 75 diagnosis of, 75 epidemiology and demographics of, 75 etiology of, 75 physical findings and clinical presentation of, 75 treatment of, 75 Falls, 77 cause of, 77 definition of, 77 diagnosis of, 77 epidemiology and demographics of, 77 etiology of, 77 history of prior, 77 physical findings and clinical presentation of, 77 risk factors for, 77 symptoms preceding, 77 treatment of, 77 Famciclovir (Famvir), 97 Famotidine, 226 Fatigue chronic, 240 differential diagnosis of, 252 and dyspnea, 7 evaluation of, 315f Fatty liver, differential diagnosis of, 252 Fatty liver hepatitis. See Nonalcoholic fatty liver disease Fecal fat, quantitative, 425 Fecal incontinence, 109 clinical assessment of, 316b diagnosis of, 109

Fecal incontinence—cont’d epidemiology and demographics of, 109 etiology of, 109 evaluation of, 316f physical findings and presentation of, 109 treatment of, 109 Felty’s syndrome, 268 Femoral neck fracture, 79, 79f definition of, 79 diagnosis of, 79 epidemiology and demographics of, 79 etiology of, 79 physical findings and clinical presentation of, 79 treatment of, 79 Fenfluramine, 173 Fenofibrates, for elevated triglycerides, 138 Fenoldopam, 104 Ferritin, serum, 425 Fever of undetermined origin, 317f–318f Fexofenadine, 184 Fibrin degradation products (FDP), 33 Fibrinogen, 156 Fibroid uterus, 70 Fibromyalgia, 80 ACR criteria for classification of, 80f definition of, 80 Fibromyalgia syndrome (FS). See Fibromyalgia Fibrositis diagnosis of, 80 epidemiology and demographics of, 80 etiology of, 80 physical findings, 80 treatment of, 80 Finasteride and dutasteride, 166 Fine-needle aspiration (FNA) biopsy with thyroid cystic lesions, 210 Fistulas, 223 Flank pain, 32 Flatulence and bloating, differential diagnosis of, 252 Flecainide, antiarrhythmic drugs, 213 Flexion tendon rupture, 217 Flexion vs. resistance, 206 Flu. See Influenza Fluconazole (Diflucan), 142, 211–212 Fludarabine, treatment for CLL, 117 18-fluorodeoxyglucose (18 FDG), 121 Flutamide, 167 Fluticasone and salmeterol, advair combines, 44 Folate, 425 Folic acid, 425 Follicle-stimulating hormone (FSH), 127 Follicular conjunctivitis. See Conjunctivitis Fondaparinux (Arixtra), 208 Foot ulcers, 63 Fosamax (alendronate sodium), 128 Fracture, bone, 319f–320f Frailty. See Failure to thrive Free T4. See T4 Friedreich’s ataxia, 244 Frigidity. See Sexual dysfunction Frontotemporal dementia cognitive and behavioral features of, 56 core features, 56 genetics and family history of, 56 imaging features of, 56 main pathologic variants of, 56 neurologic features of, 56 pathology, 56 prevalence, 56 Frontotemporal lobar degeneration (FTLD), 56 FTD. See Frontotemporal dementia 5FU, 33

563

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Index Fuch’s dystrophy, 229 Functional independence measure (FIM/P1TM/P0) instrument, 550 Furosemide, 91

G -adrenergic blockers, for controlling BP and tachyarrhythmias, 5 -glutamyl transferase, 426 -glutamyl transpeptidase (GGTP), 45  glutamyltransferase (GGTP), 4 G20210A prothrombin mutation, hematologic disorders, 208 Gabapentin, 216 neurontin, 183 in treatment of pain and sleep interference, 97 GAD. See Anxiety Gait abnormality, differential diagnosis of, 252 Galantamine, acetylcholinesterase inhibitors, 58 Gallium nitrate, 33 Gallstones. See Cholelithiasis Gamma-knife radiosurgery, for trigeminal neuralgia, 216 Ganglia, space-occupying lesions, 205 Gardner syndrome, 210 Gastric cancer, 81 definition of, 81 diagnosis of, 81 epidemiology and demographics of, 81 etiology of, 81 physical findings and clinical presentation of, 81 treatment of, 81 Gastric ulcer (GU), 154 Gastritis, chronic atrophic, 81 Gastroesophageal reflux disease, 82, 116 definition of, 82 diagnosis of, 82 epidemiology and demographics of, 82 etiology of, 82 physical findings and clinical presentation of, 82 treatment of, 82 Gaucher’s disease, 268 Gefitinib (Iressa), 122 Gemcitabine, 33 General inpatient care (GIP), 475 Genital prolapse. See Uterine prolapse Genitourinary/gastrointestinal, prophylactic regimens for, 461t GERD. See Gastroesophageal reflux disease Geriatric abuse. See Abuse, elder management of, 277f Geriatric depression scale (GDS), 59, 75, 523 Geriatric home visit, problems during, 478t Germ cell tumor. See Ovarian cancer GI bacterial infections, 170 Giant cell arteritis, 83 clinical presentation and physical findings of, 83 definition of, 83 diagnosis of, 83 epidemiology and demographics of, 83 etiology of, 83 treatment of, 83 Gingko biloba, 213 Glargine, long-acting insulin, 64 Glaucoma, 230t acute angle-closure, 85f chronic open-angle definition of, 84 diagnosis of, 84

Glaucoma—cont’d epidemiology and demographics of, 84 etiology of, 84 Glenoid labral tear, 268 Glitazones, oral hypoglycemic agents, 63 Glomerular, 95 Glomerular filtration rate, 181 Glomeruli, abnormalities of, 266 Glossitis with iron deficiency, 72 Glucose, 214 fasting, 426 postprandial, 426 Glucose tolerance test, 426 Glue sniffer’s neuropathy, 88 Glycated hemoglobin, 426 Glycemic index, 63 Glycoprotein IIb receptor inhibitors, 136 Glycopyrrolate or amitryptyline, with sialorrhea, 8 GnRH agonists, 167 GnRH antagonists, 145 Goiter differential diagnosis of, 252 evaluation and management of, 321 Goodpasture’s syndrome, 179, 254, 266 Goserelin leuprolide, 167 Gout, 86 definition of, 86 diagnosis of, 86 epidemiology and demographics of, 86 etiology of, 86 evaluation of suspected, 322f physical findings and clinical presentation of, 86 treatment of, 86, 87t Grading lymphomas, classification systems for, 124t Graham Steell murmur, 130 Gram-negative organisms for nosocomial pneumonias, 162 Gram-negative rods, 41 Granulocyte colony-stimulating factor (GCSF), 123, 134 Granulocyte-macrophage colony-stimulating factor, 134 Granuloma cell tumor, 70 Graves’ acropachy, 106 Graves’ disease, 106, 248, 252 an autoimmune disease, 10 with exophthalmos, lid retraction, 106 Graves’ ophthalmopathy, 106 Grawitz tumor. See Renal cell adenocarcinoma Greater trochanteric pain syndrome. See Trochanteric bursitis Guaifenesin, an expectorant, 44 Guillain-Barré neuropathy, 263 Guillain-Barré syndrome, 29, 158, 244, 250–251, 264, 267 definition of, 88 diagnosis of, 88 epidemiology and demographics of, 88 etiology of, 88 physical findings and clinical presentation of, 88 treatment of, 88 Gyne-Moistrin cream, 127 Gynecomastia differential diagnosis of, 252 evaluation of, 323f

H H. pylori infection, 10, 26 eradication of, 155 H. pylori testing, endoscopic biopsy for, 154 Hachinski ischemic score, 509

Haemophilus influenzae and Streptococcus pneumoniae, 44 Haldol, dopamine-blocking neuroleptics, 204 Halitosis, differential diagnosis of, 252–253 Ham test, 426 Hamilton anxiety rating scale (HARS), 524 Hamman-Rich pneumonitis, 250 “Hampton’s hump”, 171 Hapatoglobin, serum, 426 Hashimoto’s disease, 425 Hashimoto’s thyroiditis, 106 HAV infection, serologic tests in, 427f Hazy cornea, 85 HDL. See High-density lipoprotein cholesterol Head and neck cancer. See Laryngeal cancer Head-up tilt test, 203f Headache differential diagnosis of, 252–253 vague, 201 Health care, durable power of attorney for, 469 Health care financing administration (HCFA), 484 Health-care workers (HCWs), immunizing agents and immunization schedules for, 452t–454t Hearing handicap inventory, 531 Hearing loss, 324b evaluation of, 324f Hearing loss, acute, differential diagnosis of, 253 Heart attack. See Myocardial infarction Heart failure, congestive. See Congestive heart failure Heart failure, diastolic, 93 definition of, 93 diagnosis of, 93 epidemiology and demographics of, 93 pathophysiology of, 93 physical findings of, 93 precipitants of diastolic heart failure of, 93 treatment of, 92 Heartburn and indigestion, differential diagnosis of, 253 treatment for, 325f Heat exhaustion and heat stroke, 94 definition of, 94 diagnosis of, 94 epidemiology and demographics of, 94 etiology of, 94 physical findings and clinical presentation of, 94 treatment of, 94 Heat illness, 94 Heat stroke, 94 Heberden’s nodes, 143 Heel pain, plantar, differential diagnosis of, 253 Hemagglutinin and neuraminidase, 112 Hematochezia/melena, 50 Hematocrit, 426 Hematologic disease, 171 Hematologic disorders, 247 Hematology Disease Site Group of Cancer Care Ontario Practice Guidelines Initiative, 132 Hematuria, 253 definition of, 95 diagnosis of, 95 differential diagnosis of for men, 253 for women, 253 epidemiology and demographics of, 95 etiology of, 95 treatment of, 95

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Index Hemiparesis, 201 and hemiplegia, differential diagnosis of, 254 Hemoccult-positive stools, 81 Hemodialysis/peritoneal dialysis, 181 Hemoglobin A1c. See Glycated hemoglobin Hemoglobin electrophoresis, 426 Hemoglobin, glycated. See Glycated hemoglobin Hemoglobin, glycosylated. See Glycated hemoglobin Hemologbin, 426 Hemolysis, drugs capable of promoting, 242 Hemolytic anemia, evaluation of patients with, 285f Hemolytic-uremic syndrome (HUS), 265, 269 Hemoptysis, 130 differential diagnosis of, 254 Hemorrhoids anatomy of internal and external, 96f definition of, 96 diagnosis of, 96 epidemiology and demographics of, 96 etiology of, 96 physical findings and clinical presentation of, 96 treatment of, 96 Henoch-Schönlein purpura, 244, 254 Heparin and aspirin, 136 with unstable angina, 13 Heparin cofactor II deficiency, hematologic disorders, 208 Hepatic encephalopathy, 56 Hepatitis A viral infection, 427 Hepatitis B surface antigen, 427 Hepatitis B vaccines, recommended doses of licensed, 460t Hepatitis B viral infection, 427 antigen and antibody in, 428f–429f Hepatitis B virus, recommended post exposure prophylaxis for, 462t Hepatitis C viral infection, antigen and antibody in, 429f Hepatitis C viral testing, 46 Hepatitis, viral, 138 acute, 327f Hepatocellular disease, diagnosing, 46 Hepatomegaly, 328f–329f clinical signs of, 35 diagnosing, 46 and splenomegaly, 123 and weight loss with GI involvement, 7 Hereditary cataracts. See Cataracts Hereditary nonpolyposis colon cancer (HNPCC), 47 Herpes zoster definition of, 97 diagnosis of, 97 epidemiology and demographics of, 97 etiology of, 97 with herpetic blisters, 29 physical findings and clinical presentation of, 97 and postherpetic neuralgia, treatment of, 330f treatment of, 97 Heterozygous mutations, 224 HgbA1c levels, 156 Hiccups, differential diagnosis of, 254 HIDA scan, 42 Hidradenitis suppurativa, 272 High blood pressure. See Hypertension High-density lipoprotein cholesterol, 430 High-dose chemotherapy (HDCT), 132 Hill’s sign, 17

Hip, osteoarthritis of, 79 Hippel-Lindau disease, 176 Histamine-2 receptor antagonists (H2RAs), 155 HIV postexposure prophylaxis regimens, basic and expanded, 466b–467b Hives. See Urticaria HMG-CoA reductase inhibitors, 214 HNPCC. See Hereditary nonpolyposis colon cancer Hoarseness, differential diagnosis of, 254 Hodgkin’s disease, 28, 123, 259–260 Holosystolic murmur, at apex, 129 Holter monitor, to evaluate atrial fibrillation, 24 Holter monitoring, for syncope, 203 Home care, 473 eligibility criteria for, 473 Home evaluation, checklist for traditional, 479f Homebound cases, examples of, according to medicare criteria, 473b Homebound criteria, 473 Homebound, definition of, 477 Homocysteine, 156 Homocystinuria, concurrent, hematologic disorders, 208 Hormonal therapy, 177 Hormone replacement therapy, during menopause, 228 Horner’s syndrome, 121 Horton’s disease. See Giant cell arteritis Hospice, 475 House calls payment for, 480 for urgent problems, 478 Housemaid’s knee (prepatellar bursitis), 37 Human papilloma virus, possible role of, 116 “Hungry bones syndrome”, 256 Huntington’s chorea, 204 Huntington’s disease, 54, 68–69, 249 Hydralazine, 91 Hydrochlorothiazide and acetazolamide, 126 5-hydroxyindole-acetic acid, Urine. See Urine 5-hydroxyindole-acetic acid Hydroxyzine, first-generation antihistamines, 226 Hypercalcemia, 176 differential diagnosis of, 254 evaluation of, 331f laboratory differential diagnosis of, 416t therapy for, 331f Hypercalciuria, metabolic defects, 95 Hypercapnia and hypoxemia, 43 and respiratory compromise after highflow oxygen therapy, 44 Hypercellular bone marrow, 134 Hyperchloremic acidosis, 240 Hypercholesteremia. See Hypercholesterolemia Hypercholesterolemia definition of, 98 diagnosis of, 98 epidemiology and demographics of, 98 etiology of, 98 physical findings and clinical presentation of, 98 treatment of, 98 Hyperdynamic apex, with palpable left ventricular lift and apical thrill, 129 Hyperglycemia, symptoms of, 62 Hyperhidrosis, differential diagnosis of, 255 Hyperhomocystinemia, hematologic disorders, 208 Hypericum, for depression, 60

Hyperkalemia diagnostic approach to, 332f differential diagnosis of, 255 evaluation and treatment of, 333f Hyperlipidemia, 138 medications to, 138 Hypermagnesemia, causes of, 432 Hypernatremia, evaluation and treatment of, 334f Hypernephroma. See Renal cell adenocarcinoma Hyperosmolar coma, 65 definition of, 100 diagnosis of, 100 etiology of, 100 physical findings and clinical presentation of, 100 treatment of, 100 Hyperosmolar nonketoic state. See Hyperosmolar coma Hyperparathyroidism, 224 definition of, 101 diagnosis of, 101 epidemiology and demographics of, 101 etiology of, 101 physical findings and clinical presentation of, 101 with secondary hyperparathyroidism, 254 treatment of, 101 Hyperprolactinemia, 71 Hypertension, 103 definition of, 103 diagnosis of, 103 epidemiology and demographics of, 103 etiology of, 103 physical findings and clinical presentation of, 103 and proteinuria, management of, 95 secondary causes of, 335f treatment of, 103 Hyperthermia, 94 Hyperthyroidism, 336f definition of, 106 epidemiology and demographics of, 106 etiology of, 106 physical findings and clinical presentation of, 106 treatment of, 107 Hyperuricemia, 86 and gout, 86 secondary, 86 Hyperuricosuria, metabolic defects, 95 Hypocalcemia, 255 evaluation of, 337f laboratory differential diagnosis of, 417t Hypocapnia, differential diagnosis of, 256 Hypogammaglobulinemia in CLL, 117 and LDH, 117 Hypogonadism, laboratory evaluation of, 338f Hypogranular and agranular neutrophils, 134 Hypokalemia diagnostic approach of, 339f differential diagnosis of, 256 Hypomagnesemia causes of, 432 differential diagnosis of, 256 Hypometabolism, on SPECT or PET, 54 Hyponatremia, 188 dilutional, 45 evaluation and treatment of asymptomatic, mild, 340f Hypophosphatemia, diagnostic workup of, 341f Hypotension, 342f postural, differential diagnosis of, 256

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Index Hypothyroidism, 108, 343f definition of, 108 diagnosis of, 108 as endocrine disorders, 56 epidemiology and demographics of, 108 etiology of primary, 108 secondary, 108 tertiary, 108 and hyperthyroidism, 71 physical findings and clinical presentation of, 108 treatment of, 108 Hypovolemia, perfusion caused by, 178 Hypoxia and hypoglycemia, 52 Hysterical paralysis, 88

I Ibandronate, 148 IBD. See Inflammatory bowel disease Ibritumomab tiuxetan (Zevalin), 123 IBS. See Irritable bowel syndrome Ibuprofen, 218 ICSI healthcare guidelines, 294f, 296f IDDM (insulin-dependent diabetes mellitus), 62 Idiopathic hypertension. See Hypertension Idiopathic hypertrophic subaortic stenosis (IHSS), 246 Idiopathic pulmonary hemosiderosis, 9 Idiopathic thrombocytopenic purpura (ITP), 269 Idopathic facial paralysis. See Bell’s palsy Imiquimod (Aldara), 28 Immunoelectrophoresis (IEP), 132 Immunotherapy, 177 Impingement syndrome. See Rotator cuff syndrome Impotence. See Erectile dysfunction; Sexual dysfunction Infarct site of, 137 size of, 137 Inflammatory bowel disease (IBD), 45, 66, 115, 170 Influenza antiviral medications, for treatment and prophylaxis, 462t definition of, 112 diagnosis of, 112 epidemiology and demographics of, 112 etiology of, 112 physical findings and clinical presentation of, 112 treatment of, 112 INH and rifampin, 220 Inhaled aerosolized prostacyclin, 174 Insomnia definition of, 113 epidemiology and demographics of, 113 etiology of, 113 synonyms of, 113 Insomnia, differential diagnosis of, 257 Inspiration, abdominal and diaphragmatic movement on, 22 Instrumental activities of daily living (IADL), 471, 477, 535 Insulin, types of, 65t International normalized ratio (INR), 430 International Prostate Symptom Score (I-PSS), 30t International travel, vaccinations for, 459t Interpersonal therapy (IPT), 60 Interstitium, abnormalities of, 266 Intestinal metaplasia of lower esophagus. See Barrett’s esophagus

Intestinal pseudoobstruction, differential diagnosis of, 257 Intraarterial tPA, 199 Intracapsular fracture. See Femoral neck fracture Intracerebral hemorrhage, 197f Intranasal corticosteroid therapy, for allergic rhinitis, 184 Intravascular hemolysis, 9 Intravenous pyelography (IVP), 176 Ipratropium (Atrovent), 44 Iridotomy, 85 Irinotecan (Camptosar), 48 Iron-binding capacity, 430 Iron deficiency anemia. See Anemia, Iron deficiency Iron deficiency, reticulocyte hemoglobin, screening test for, 9 Iron therapy, with oral preparations, or malabsorption, 9 Irritable bowel syndrome, 115, 170 criteria for diagnosis of, 115 definition of, 115 diagnosis of, 115 epidemiology and demographics of, 115 etiology of, 115 physical findings and clinical presentation of, 115 treatment of, 115 Irritable colon. See Irritable bowel syndrome Ischemia myocardial, 12, 16, 93 silent, 12 Ischemic colitis, 66 nonocclusive, 257 Ischemic stroke, risk factors for, 197b Isosorbide, 91 Itching, 183 Itraconazole, 141–142 Itraesophageal bile reflux, on pathogenesis, 26 IV aminophylline administration, 44 IV mannitol, 85 Ivermectin, an antihelminitic agent, 186 Ivy method, modified, 415

J Jackhammer operators, 248 Jaundice and hepatobiliary disease, evaluation of, 344f JNC 7, 103 Jod-Basedow phenomenon, 106 Joint effusion, 345 Joint swelling, differential diagnosis of, 258 Jugular vein thrombus, 261

K K-Y jelly, 127 Kaposi’s sarcoma, 68, 207 Katz index, of activities of daily living, 536 Kayser-Fleischer rings, 45, 74 Kegel, pelvic muscle exercise, 111 Kennedy’s disease, 8 Keratitis, corneal disease, 85 Keratolysis exfoliativa, 212 Kerley B lines, 130 Ketoconazole (Nizoral), 211 “Keyhole” distribution, 118 Kidney biopsy, 181 Kidneys, sonographic evaluation of, 181 Klinefelter’s syndrome, 252 Klippel-Trenaunay syndrome, 255 Klüver-Bucy syndrome, 56 Knee pain, differential diagnosis of, 258 Krabbe’s disease, 264

L L-dopa, treatment with, 204 Lactate dehydrogenase, 430 Lactose intolerance, 66 Lacunar disease, 214 Lambert-Eaton syndrome, 260 Lamictal, 159 Lamotrigine/oxcarbazepine, 188 Langerhans’ cell histiocytosis, 245 Langhans’ giant cells, 219 Lanolin, topical emollients, 196 Lanoplase and heparin bolus plus infusion, 136 LAP score. See Leukocyte alkaline phosphatase Laparoscopic cholecystectomy for common bile duct stones, 42 for residual gallbladder stones, 42 Laryngeal cancer definition of, 116 diagnosis of, 116 epidemiology and demographics of, 116 etiology of, 116 physical findings and clinical presentation of, 116 treatment of, 116 Laryngeal papilloma, 116 Laryngitis, 116 Laser iridotomy, 85 Lateral spinal stenosis. See Spinal stenosis Laurence-Moon-Biedl syndrome, 273 LDH. See Lactate dehydrogenase LDL. See Low-density lipoprotein cholesterol Lead, heavy metal poisoning, 88 Left ventricular hypertrophy (LVH), 12, 103 Leg cramps, nocturnal, differential diagnosis of, 259 Leg pain with exercise, 259 Leg ulcers, 347f differential diagnosis of, 259 Lenalidomide, an analogue of thalidomide, 134 Leukemia, chronic lymphocytic. See Chronic lymphocytic leukemia Leukocyte alkaline phosphatase, 431 Leukocyte count. See Complete blood count Levodopa/carbidopa (Sinemet), 183 Levodopa therapy, 153 Levofloxacin, 49 Levothyroxine, 74 Lichen planus, 141 Lichen sclerosus, 118, 118f, 233, 272 definition of, 118 diagnosis of, 118 epidemiology and demographics of, 118 etiology of, 118 physical findings and clinical presentation of, 118 treatment of, 118 Lidocaine antiarrhythmic drugs, 213 in relieving postherpetic neuralgia, 97 Life expectancy definitions and concepts of, 481 by race, sex, and age, 481t Ligament tissue, crystal deposition in, 169 Lindane lotion, 186 Linitis plastica. See Gastric cancer Lipase, 431 Lipid-lowering drugs, use of, 181 with coronary heart disease, 14 with hyperlipidemia, 14 Lipomas, space-occupying lesions, 205 Lipoprotein cholesterol, high-density. See High-density lipoprotein cholesterol

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Index Lipoprotein cholesterol, low-density. See Low-density lipoprotein cholesterol Lipoprotein metabolism, drugs affecting, 99f Lithium, 74 for treatment of MDE, 60 Liver biopsy, in evaluating hepatic filling defects, 46 Liver disease decreased glucose with, 45 toxin or drug-induced, 138 Liver function test elevations, 348f Loop diuretics and oxygen, for CHF, 24 Loperamide, for diarrhea, 115 Loratadine non-sedating antihistamines, 226 second-generation oral antihistamines, 184 Loss of consciousness (LOC), 202 Loss of libido, 71 Low-density lipoprotein cholesterol, 431 Low vision. See Vision loss Löwenstein-Jensen, 219 Lower esophageal sphincter (LES), 82 Lower lumbar and sacral radiculopathy, diagnosis of, 119t Lower motor neurons (LMN), 8 Lower urinary tract, 95 Lumbar disk syndrome, 79, 119 definition of, 119 diagnosis of, 119 epidemiology and demographics of, 119 etiology of, 119 physical findings and clinical presentation of, 119 Lumbar puncture, 187 during vasculitis/amyloid, 158 Lumbar spine bone mineral density, history of, 146f Lumen, “swatooth” appearance of, 66 Lung cancer, 121 treatment of small cell, 122 Lung, metastatic carcinoma to, 121 Lung neoplasms, primary, 121 definition of, 121 diagnosis of, 121 epidemiology and demographics of, 121 etiology of, 121 physical findings and clinical presentation of, 121 treatment of, 122 Lung transplantation and heart-lung transplantation, 174 Lupus anticoagulant. See Circulating anticoagulant hematologic disorders, 208 Lutein, with antioxidants, 231 Luteinizing hormone (LH), 127 Lyme disease antibody titer, 68, 88, 211, 263, 431 Lymphadenopathy, 259 axillary, 349f cervical, 350f generalized, 351 Lymphocytes, 431 Lymphoma, 59

M M spike, 132 Macrocytic anemia, differential diagnosis of, 283f Macroglossia, with GI involvement, 7 Macular degeneration definition of, 125 diagnosis of, 125 epidemiology and demographics of, 125 etiology of, 125 treatment of, 125

Magnesium, 432 Magnetic resonance direct thrombus imaging (MRDTI), for evaluation of DVT, 208 Magnetic resonance imaging (MRI) to assess extent of prostate cancer, 166 and MRA, 214 Major depressive episodes (MDE), 59 Malabsorption with GI involvement, 7 suspected, 352b, 352f Male erectile disorder. See Erectile dysfunction Mallucci syndrome, 255 Manganese, as toxins, 152 Marfan’s syndrome, 11, 16, 131, 145 Marinol, 76 Massachusetts Male Aging Study, 71 May-Hegglin anomaly, 269 McCall culdoplasty, 228 MCI. See Mild cognitive impairment MDD. See Depressive disorder, major MDEs and manic episodes, 59 MDS. See Myelodysplastic syndrome Mean corpuscular volume, 4 cause of decreased, 433t cause of increased, 433t Medial collateral ligament (MCL), 38 Medicare and Medicaid, 484 Medicare home care eligibility criteria, clinical vignettes related to, 474 Medicare Part A coverage benefits of, 484t deductibles, co-pays, and limitations, 484t items not in, 485t Medicare Part B coverage components of, 485t items not in, 485t premiums, deductibles, and co-pays for, 485t Medroxyprogesterone acetate (Provera), 128 Megaloblastic anemia, 10 Megestrol, 76 Meigs’ syndrome, 244, 264 Melanoma of vulva. See Vulvar cancer Melkersson-Rosenthal syndrome, 29 Melphalan and prednisone, 132 Memantine, 58 Meniere’s disease, 126 definition of, 126 diagnosis of, 126 epidemiology and demographics of, 126 etiology of, 126 physical findings and clinical presentation of, 126 treatment of, 126 Meniere’s syndrome. See Meniere’s disease Menisci, crystal deposition in, 169 Menopause definition of, 127 diagnosis of, 127 epidemiology and demographics of, 127 physical findings and clinical presentation of, 127 treatment of, 127 Mental status assessment (MSA), 57 Mental status change, algorithm for evaluation of, 303f Mesenteric ischemia, nonocclusive, differential diagnosis of, 260 Metabolic acidosis, 414 Metabolic cataracts. See Cataracts Metastatic carcinoma, 123 Metastatic neoplasms, differential diagnosis of, 260 Metformin (Glucophage), 63, 138, 140 to decrease hepatic glucose output, 63 Methimazole (Tapazole), 107

Methionine, 414 Methyl tert-butyl ether (MTBE), 42 Metoclopramide, 82, 204 Metoprolol (Lopressor), 136 Metronidazole, 170 Metvix (methyl aminolevulinate cream), 28 MI. See Myocardial infarction Michigan alcoholism screening test, 505–506 Miconazole cream (Monistat-Derm), 211 Microcytic anemia, differential diagnosis of, 284f Micturition, 202 Midazolam for managing DTs, 5 a short-acting sedative, 24 Migraine, 214 Mild cognitive impairment diagnostic criteria for, 57 vs. mild dementia, 57 normal aging vs., 57 Mini-Cog scoring algorithm, 510 Mini-mental state examination (MMSE), 511 modified, 512 severe, 513–514 Minimally invasive direct coronary artery bypass (IDCAB), 14 Minimum data set (MDS), 487 Mirizzi’s syndrome, 248 Mirtazapine, 76 Misoprostol therapy, for prevention of NSAID-induced gastric ulcers, 155 Missoula-Vitas quality of life index (MVQOLI), 547 Mitoxantrone, 33 Mitral click murmur syndrome. See Mitral valve prolapse Mitral insufficiency. See Mitral regurgitation Mitral regurgitation, 129–131, 246 definition of, 129 diagnosis of, 129 epidemiology and demographics of, 129 etiology of, 129 physical findings and clinical presentation of, 129 treatment of, 129 Mitral stenosis, 246 definition of, 130 diagnosis of, 130 epidemiology and demographics of, 130 etiology of, 130 physical findings and clinical presentation of, 130 treatment of, 130 Mitral valve prolapse, 131, 246 as asymptomatic, 131 definition of, 131 diagnosis of, 131 epidemiology and demographics of, 131 etiology of, 131 physical findings and clinical presentation of, 131 treatment of, 131 Moderate stasis dermatitis with hyperpigmentation, 196f Mohs’ micrographic surgery, 28, 195 Monoamine oxidase inhibitors (MAOIs), 60 Monoclonal gammopathy of undetermined significance (MGUS), 132, 158 Mononeuropathy, 158 differential diagnosis of, 260 multiplex, 158 Monotherapy, 64t Morpheaform, 28 Motor neuron disease (MND), 56 MR. See Mitral regurgitation MS. See Mitral stenosis Mucositis, 68

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Index Mucous membrane exposures and nonintact skin exposures, recommended HIV postexposure prophylaxis for, 463t Multifocal motor neuropathy (MMN), 8 Multinodular goiter, 107 Multiple myeloma, 132 definition of, 132 diagnosis of, 132 epidemiology and demographics of, 132 physical findings and clinical presentation of, 132 treatment of, 132 Munchausen’s and papillary necrosis, 222 Murmur diastolic, 354 systolic, 355, 355f Muscle cramps and aches, evaluation of, 356f Muscle weakness, 357f differential diagnosis of, 260 lower motor neuron vs. upper motor neuron, 260 MVP. See Mitral valve prolapse Mycobacterial and fungal diseases, 121 Mycobacterium tuberculosis, 219 Myelodysplastic syndrome, 134, 358f definition of, 134 diagnosis of, 134 epidemiology and demographics of, 134 etiology of, 134 French-American-British classification, 134 physical findings and clinical presentation of, 134 treatment of, 134 Myeloma chemotherapeutic agents in multiple, 132 multiple, 353f Myelopathy with polyradiculopathy, 8 Myocardial infarction, 135 analgesia for, 136 ejection fraction after, 137 definition of, 135 diagnosis of, 135 epidemiology and demographics of, 135 etiology of, 135 non-ST elevation, 135 physical findings and clinical presentation of, 135 with pump failure, 202 ST elevation, 135 treatment of, 135 Myocardial ischemia differential diagnosis of, 261 stress echocardiogram or radionuclide testing in detection of, 13 suspected, 359f Myofascial pain syndrome. See Fibromyalgia Myxedema. See Hypothyroidism

N N-telopeptide, 148 Naftifine cream (Naftin), 211–212 Naproxen, 218 Narcotic analgesics, 159 Narrow-angle glaucoma. See Primary closedangle glaucoma Narrow complex tachycardia, evaluation and management of, 390f Nasal or throat swab, 112 Nasal oxygen, for acute MI, 136 Nascobal (intranasal cyanocobalamin), 10 National Cancer Institute Consensus guidelines, for universal screening for HNPCC, 48 National Council on Alcoholism criteria, screening instrument for alcoholism, 4

National Pressure Ulcer Advisory Panel (NPUAP) staging system, 542 Nausea and vomiting, 132, 224, 261 Neck mass, differential diagnosis of, 261 Neck pain, differential diagnosis of, 261 Necrobiosis lipoidica diabeticorum, 63 Necrotizing pneumonia, 220 Nedocromil (Tilade), 22 Nephrectomy, role of, 176 Nephrolithiasis, evaluation of suspected, 360f Nephrotic syndrome, systemic disorders, 108 Nerve conduction velocity (NCV), 194 Nervous system, defects in peripheral, 240 Nesiritide, direct vasodilating drugs, 91 Neurogenic bladder, differential diagnosis of, 262 Neurologic deficit focal, 262 multifocal, 262 Neuromuscular diseases causing hypoventilation, 173 Neurontin, 74, 159 Neuropathic pain, treatment of, 159 Neuropathies, painful, 262 Neutropenia algorithm for evaluation of, 361f drugs causing, 434t New York Heart Association (NYHA) functional classification, 369t on heart failure, 90 NHL. See Non-Hodgkin lymphoma Nicotine, 74 NIDDM (non-insulin-dependent diabetes mellitus), 62 Nilutamide, as antiandrogens, 167 Nitrates for acute MI, 135 cause venodilation and vascular smooth muscle, 13 Nitroprusside, in hypertensive encephalopathy, hypertension and intracranial bleeding, 104 Nocturnal leg cramps with/without peripheral artery disease, 183 Nocturnal myoclonus. See Restless legs syndrome Non-Hodgkin lymphoma, 259–260 definition of, 123 diagnosis of, 123 epidemiology and demographics of, 123 physical findings and clinical presentation of, 123 treatment of, 123 working formulation of, 123 Non-ST elevation MI. See Myocardial infarction Nonalcoholic fatty liver disease, 138 definition of, 138 diagnosis of, 138 epidemiology and demographics of, 138 etiology of, 138 physical findings and clinical presentation of, 138 treatment of, 138 Nonalcoholic steatohepatitis. See Nonalcoholic fatty liver disease Nonarticular rheumatism. See Fibromyalgia Nonbacterial prostatitis and prostatodynia, 168 Noncardiac surgical procedures, cardiac risk stratification for, 540t Noninvasive methods, for assessing myocardial viability, 13 Nonketoic hyperosmolar syndrome. See Hyperosmolar coma Nonpharmacologic sleep protocol, use of, 52

Nonsteroidal antinflammatory drugs. See NSAIDs Norepinephrine, 434 Normal pressure hydrocephalus (NPH), 253 Norton scale/Braden scale, 234 Norwegian/crusted scabies, 186 Nottingham health profile (NHP), 549 NPH, long-acting insulin, 64 NSAIDs, 205, 218, 255 conservative therapy with, 194 for gout, 169 5´-nucleotidase, 434 Numeric rating scale, 534 Nummular eczema, 186, 196 Nursing home attending physician role, 492 medical director role, 494 physician responsibilities in, 488t residents, 487 staffing, 487 visits, 489, 489t Nutritional support, decision approach for type and route of, 362f Nystagmus, differential diagnosis of, 262

O Obesity, 139 with anserine bursitis, 38 in black and Hispanic women, 139 cause of, 139 definition of, 139 diagnosis of, 139 epidemiology and demographics of, 139 etiology of, 139 physical findings and clinical presentation of, 139 in primary care setting, 140 treatment of, 139 Obstructive sleep apnea-hypopnea syndrome, 192 Occipital lobe infarct, 197f Occupational blood exposures, management of, 465b Occupational exposure management resources, 464b Occupational therapy, 497 Ocular Hypertension Treatment Study, 84 Odynophagia. See Dysphagia Ofloxin (antibiotic drops), 49 Ogilvie’s syndrome, 257 Onychomycosis definition of, 141 diagnosis of, 141 distal subungual, 141f epidemiology and demographics of, 141 etiology of, 141 physical findings and clinical presentation of, 141 superficial white, 141f treatment of, 141 Open-angle glaucoma definition of, 84 diagnosis of, 84 epidemiology and demographics of, 84 etiology of, 84 Ophthalmic vessels, fluorescein angiogram of, 83 Ophthalmoplegia, differential diagnosis of, 263 Opioids, 183 Optic nerve cupping, 85 Optical coherence tomography (OCT), 125 Oromandibular dystonia. See Dystonia Oropharyngeal dysphagia. See Dysphagia Orthostasis, 77 Oseltamivir, 112

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Index Osmolality, 434 Osteitis deformans. See Paget’s disease Osteoarthritis, 80 definition of, 143 diagnosis of, 143 of DIP joints, 143f epidemiology and demographics of, 143 etiology of, 143 physical findings and clinical presentation of, 143 treatment of, 143 Osteoarthrosis. See Osteoarthritis Osteomyelitis, 144f chronic, 144 contiguous, 144 definition of, 144 diagnosis of, 144 epidemiology and demographics of, 144 etiology of, 144 hematogenous, 144 treatment of, 144 vertebral, 144 Osteonecrosis. See Aseptic necrosis Osteoporosis definition of, 145 diagnosis of, 145 epidemiology and demographics of, 145 etiology of, 145 physical findings and clinical presentation of, 145 prevention and treatment of postmenopausal, 147t primary, 145 secondary, 145 treatment of, 146 workup for cause of, 147t Ovarian cancer definition of, 149 diagnosis of, 149 epidemiology and demographics of, 149 etiology of, 149 physical findings and clinical presentation of, 149 treatment of, 149 Overactive bladder. See Urinary incontinence Overweight. See Obesity Oxaliplatin, 48 Oxybutynin, 111 Oxymetazoline, 184

P P. aeruginosa infection, 163 PAD. See Peripheral arterial disease Paget’s disease, 101, 145, 194, 232, 253, 255, 257–258, 272 definition of, 150 diagnosis of, 150 epidemiology and demographics of, 150 etiology of, 150 physical findings and clinical presentation of, 150 treatment of, 150 Pain control, 363, 363f Pain/paresthesias, distribution of, 39f “Painful arc”, 185 Painful arc syndrome. See Rotator cuff syndrome Painful heel spur. See Plantar fasciitis Painful heel syndrome. See Plantar fasciitis Palliative care, 498 components of, 498 Pallidal (globus pallidus interna), 153 Palpitations, differential diagnosis of, 263 “Pancoast” tumor, 121 Pancreatic cancer definition of, 151

Pancreatic cancer—cont’d diagnosis of, 151 epidemiology and demographics of, 151 etiology of, 151 physical findings and clinical presentation of, 151 treatment of, 151 Pancreatitis, chronic, 115 Pancytopenia with bone marrow replacement, 263 with hypocellular bone marrow, 263 with normal or increased cellularity of hematopoietic origin, 263 Papaverine, intracavernous injection, 189 Papillary conjunctivitis. See Conjunctivitis Papillary necrosis, 223 Paracentesis fluid, 434 Paralysis agitans. See Parkinson’s disease Paralysis, hysterical, 88 Parapneumonic effusion-empyema, 163 Parathyroid gland, percutaneous ethanol injection into, 101 Parathyroid hormone-like protein (PLP), 101 Parathyroid hormone (PTH), 101 Paresthesias, differential diagnosis of, 263 Parkinson’s disease, 50, 59, 68–69, 74, 113, 202, 249, 262, 365 definition of, 152 diagnosis of, 152 epidemiology and demographics of, 152 etiology of, 152 physical findings and clinical presentation of, 152 treatment of, 152 Parkinson’s pugilistica, 152 Paroxysmal nocturnal dyspnea (PND), 90 Paroxysmal nocturnal hemoglobinuria, 9 Partial thromboplastin time, 434 and PTT, 214 Patient Self-Determination Act of 1991, 469 PCP, 15 PE. See Pulmonary embolism Pectus excavatum, 131 Pelvic mass approach for, 366f differential diagnosis of, 263 Pelvic organ prolapse. See Uterine prolapse Pelvic organ prolapse quantification (POPQ), 227b Pelvis, frontal radiograph of, 150f Penile damage, 71 Pentolaine, intracavernous injection, 189 Pentoxifylline/yohimbine, oral medications, 71 Pentoxyphylline (Trental, Pentoxil), 156 Peptic esophagitis. See Gastroesophageal reflux disease Peptic ulcer disease, 26, 82 definition of, 154 diagnosis of, 154 epidemiology and demographics of, 154 etiology of, 154 physical findings and clinical presentation of, 154 treatment of, 154 Percutaneous coronary intervention (PCI), 14 Percutaneous electrical nerve stimulation (PENS), 119 Percutaneous injuries, recommended HIV postexposure prophylaxis for, 463t Percutaneous transhepatic cholangiography (PTC), 46 Percutaneous transvenous mitral valvotomy (PTMV), 130 Pergolide, 183 Pericardial effusion, differential diagnosis of, 263

Periodic health examination, 447t Periodic limb movement disorder (PLMD), 183 Perioperative cardiovascular risk, clinical predictors of, 539t Peripheral arterial disease definition of, 156 diagnosis of, 156 epidemiology and demographics of, 156 etiology of, 156 physical findings and clinical presentation of, 156 treatment of, 156 Peripheral nerve dysfunction definition of, 158 diagnosis of, 158 etiology of, 158 physical findings and clinical presentation of, 158 treatment of, 159 Peripheral neuropathy, 158 approach for, 367f Pes anserinus bursitis. See Bursitis, anserine Peutz-Jeghers syndrome, 47 Peyronie’s disease, 189, 251 Peyronie’s fibrosis, 71 PH, blood, 435 Phalen’s test, 39 Phenothiazines, 151 Phenytoin, therapeutic agent, 188 Phlebitis. See Thrombophlebitis, superficial Phosphatase, acid. See Acid phosphatase Phosphatase, alkaline. See Alkaline phosphatase Phosphate, serum, 435 Phosphorus and magnesium, 5 Photodynamic therapy, 28 Pick’s disease, 249 Piles. See Hemorrhoids Pilocarpine, 85 Pink puffers (emphysema), 43 Pioglitazone and rosiglitazone, 63 “Pistol shot” femorals, 17 Plantar fasciitis definition of, 160 diagnosis of, 160 epidemiology and demographics of, 160 etiology of, 160 injection site for, 160f physical findings and clinical presentation of, 160 treatment of, 160 Plasma methylmalonic acid (P-MMA), 10 Platelet count, 435 Pleural effusion and empyema, evaluation, common etiologies, and management of, 368f Pleural effusions, differential diagnosis of, 263 Plummer’s disease, 106 Plummer-Vinson syndrome, 72 Pneumococcal infection for communityacquired pneumonias, 162 Pneumonia, 121. See also Pneumonia, aspiration community-acquired anaerobic aspiration, 161 community-acquired aspiration, 161 hospital-acquired aspiration, 161 Pneumonia, aspiration definition of, 161 diagnosis of, 161 epidemiology and demographics of, 161 etiology of, 161 physical findings and clinical presentation of, 161 treatment of, 161

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Index Pneumonitis, viral, 121 Polymerase chain reaction (PCR), 219 Polymyalgia rheumatica, 80, 164 definition of, 164 diagnosis of, 164 differential features in, 164t epidemiology and demographics of, 164 etiology of, 164 physical findings and clinical presentation of, 164 treatment of, 164 Polymyositis, 164 Polyneuropathy, 158 differential diagnosis of, 264 drug-induced, 264 vs. mononeuropathy multiplex, 158 symmetric, 264 Polyuria, differential diagnosis of, 265 Portal hypertension, differential diagnosis of, 265 Positron emission tomography (PET), 121 Postinfectious polyneuritis. See Guillain-Barré syndrome Postmenopausal vaginal bleeding, differential diagnosis of, 265 Potassium hydroxide (KOH), 212 Potassium, serum, 435 Povidine-iodine (betadine), 49 PPH. See Pulmonary hypertension Practice agreement, 492f Prader-Willi syndrome, 273 Pramipexole, 183 Prazosin, 31 Prednisone and melphalan, 132 Preleukemia. See Myelodysplastic syndrome Premethrin 5% cream (Elimite), 186 Preoperative cardiac assessment, 538 Presbyopia, 230t Presenile dementia. See Dementia Pressure sore risk, Braden scale for predicting, 541 Pressure sores. See Wounds Pressure ulcer scale for healing (PUSH), 544–545 Pretibial myxedema, with Graves’ disease, 106f Preventive health screening, discontinuation considerations in, 468t Primary closed-angle glaucoma, 85 definition of, 85 diagnosis of, 85 epidemiology and demographics of, 85 etiology of, 85 physical findings and clinical findings of, 85 Primary hyperparathyroidism as asymptomatic, 101 as symptomatic, 101 Primidone, 74 Probenecid, 86 Prochlorperazine, 204 Progesterone challenge test, 127 Prophylactic regimens for dental, oral, respiratory tract, or esophageal procedures, 461t Propranolol, 74 Propylthiouracil (PTU), 107 Prostaglandin E1, 189 Prostate cancer androgen-deprivation therapy for metastatic, 167 assessment and treatment for, 370f definition of, 166 diagnosis of, 166 epidemiology and demographics of, 166 measurement of PSA in, 166

Prostate cancer—cont’d physical findings and clinical presentation of, 166 treatment of, 166–167 Prostate-specific antigen age-specific reference ranges for, 436t factors affecting serum, 436t Prostatic acid phosphatase (PAP), 166 Prostatic hypertrophy. See Benign prostatic hyperplasia Prostatitis, 168 definition of, 168 diagnosis of, 168 epidemiology and demographics of, 168 etiology of, 168 physical findings and clinical presentation of, 168 treatment of, 168 Protein C deficiency, hematologic disorders, 208 Protein electrophoresis, 437 Protein S deficiency, hematologic disorders, 208 Protein, serum, 437 Proteinuria, 372f Prothrombin time, 438 Proton pump inhibitors (PPI), 154–155 Protoporphyrin, 438 Pruritus, 212 differential diagnosis of, 265 evaluation of generalized, 373 PSA. See Prostate-specific antigen Pseudoclaudication, 194 Pseudogout, 169 definition of, 169 diagnosis of, 169 epidemiology and demographics of, 169 etiology of, 169 physical findings and clinical presentation of, 169 treatment of, 169 Pseudohypoparathyroidism (PHP), 256 Pseudomembranous colitis, 170 definition of, 170 diagnosis of, 170 epidemiology and demographics of, 170 etiology of, 170 physical findings and clinical presentation of, 170 treatment of, 170 Pseudomembranous conjunctivitis. See Conjunctivitis Pseudomembranous plaques with coronoscopy, 170f Pseudomonas aeruginosa, 144 Psoriasis, 141 Psychiatric advance directives (PAD), 469 Psychogenic rheumatism. See Fibromyalgia Psychotic illness, acute, 52 PT. See Prothrombin time PTT. See Partial thromboplastin time PTU and methimazole, 107 PUD. See Peptic ulcer disease “Pull test”, 152 Pulmonary angiogram, for suspicious V/Q scans, 174 Pulmonary arterial hypertension, progressive, 174f Pulmonary edema, acute, 130 Pulmonary embolism, 374f definition of, 171 diagnosis of, 171 epidemiology and demographics of, 171 etiology of, 171 inferior vena cava filter to prevent, 209

Pulmonary embolism—cont’d physical findings and clinical presentation of, 171 treatment of, 172 Pulmonary eosinophilic syndromes, 163 Pulmonary function testing (PFT), 43, 192 Pulmonary hypertension definition of, 173 diagnosis of, 173 epidemiology and demographics of, 173 etiology of, 173 physical findings and clinical presentation of, 173 treatment of, 174 Pulmonary lesions, differential diagnosis of, 265 Pulmonary nodule, 375f solitary, differential diagnosis of, 265 Pulmonary thromboembolism. See Pulmonary embolism Pulmonary venous disease, 173 Pulmonic regurgitation (PR), 247 Pulmonic stenosis (PS), 246 Pulse oximetry, 162 Pupillary block glaucoma. See Primary closed-angle glaucoma Purified protein derivative (PPD), 219 Purpura, differential diagnosis of, 265 Purulent conjunctivitis. See Conjunctivitis Pyrophosphate arthropathy. See Calcium pyrophosphate dehydrate

Q QT interval prolongation, differential diagnosis of, 265 Quality indicators (QIS), 487 examples of nursing home, 488t Quality of life, 500 components of, 500 differential diagnosis of, 500 evaluation of, 500 Quantitative computed tomography (QCT), 146 Quantitative ultrasound (QUS), 146 Quetiapine, atypical antipsychotic, 152 Quincke’s pulse, 17

R “Raccoon-eyes”, 7 Radiation therapy, 28 Radiation therapy, complications of, 73 Radioactive iodine (RAI), with Graves’ disease, 107 Radioiodine, with hyperthyroidism, 107 Ramelteon, 114 Ramsay-Hunt syndrome, 29 Ranitidine, 226 Rapid eye movement (REM), 56 Rasmussen’s aneurysm, 219 Raynaud’s phenomenon, 255 RDW. See Red blood cell distribution width Reactive airways disease. See Asthma Rectal pain, differential diagnosis of, 266 Red blood cell and angina, 9 count, 438 distribution width, 9, 438 transfusion of packed, 9 Red eye acute, 376 differential diagnosis of, 266 “Red eye”/“Pink eye”. See Conjunctivitis Reflux esophagitis. See Gastroesophageal reflux disease Refsum’s disease, 264

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Index Rehabilitation, 502 process for, 502 professionals, 502 Reiter’s syndrome, 194, 244, 250 Renal biopsy with intrinsic renal failure, 179 Renal cell adenocarcinoma conventional vs. TNM staging classification, 177t definition of, 176 diagnosis of, 176 epidemiology and demographics of, 176 etiology of, 176 physical findings and clinical presentation of, 176 Renal cell carcinoma, large, 176f Renal colic. See Urolithiasis Renal failure, 59 chronic, 181 definition of, 178, 181 diagnosis of, 178, 181 epidemiology and demographics of, 178, 181 etiology of, 178, 181, 377f physical findings and clinical presentation of, 178, 181 treatment of, 179, 181 postrenal causes, 266 prerenal causes, 266 and sepsis, 180 serum and radiographic abnormalities in, 178t Renal mass, evaluation of, 378f Renovascular hypertension (RVH), treatment of, 104 Reserpine, 204 Resident assessment protocols (RAPs), 487 Respiration, defects in muscles of, 240 Respiratory center, depression of, 240 Respiratory distress, 379f Respiratory disturbance index (RDI), 192 Respiratory failure, hypoventilatory, 266 Respiratory system disease, diagnosis of, 311f Restless legs syndrome definition of, 183 diagnosis of, 183 epidemiology and demographics of, 183 etiology of, 183 physical findings and clinical presentation of, 183 treatment of, 183 Restriction fragment length polymorphism (RFLP), 219 Retinal detachment, 230t Retinal vein occlusion, 230t Retropulsion, 152 Reye’s syndrome, 261 Rheumatic fever, 130 Rheumatoid arthritis, 7, 19f–20f, 80, 164 Rhinorrhea, approach to, 380f Rhinosinusitis, allergic, 184 definition of, 184 diagnosis of, 184 epidemiology and demographics of, 184 physical findings and clinical presentation of, 184 treatment of, 184 Rifapentine, 221 Riluzole (Rilutek), 8 Ringing in the ear(s). See Tinnitus Ringworm. See Tinea corporis Ringworm of nails. See Onychomycosis Risedronate, 148 Risperidone and olanzapine, 58 Rituximab vs. CD20 B-cell lymphoma, 123 Rivastigmine, 58 RNP. See Extractable nuclear antigen Ropirinole, 183

Rosenbaum pocket vision screener, 556 Rotator cuff lesions, 185f Rotator cuff syndrome definition of, 185 diagnosis of, 185 epidemiology and demographics of, 185 etiology of, 185 physical findings and clinical presentation of, 185 treatment of, 185

S S3 gallop, 90 S. aureus (methicillin-resistant), 144 Salmonella (in sickle cell disease), 144 Scabies definition of, 186 diagnosis of, 186 epidemiology and demographics of, 186 etiology of, 186 physical findings and clinical presentation of, 186 treatment of, 186 in wet mount preparation, 186f SCC. See Squamous cell carcinoma Schatzki’s ring, 26, 68 Schilling test, 10 Schirmer’s test, 191 Scleritis, 85 Scrotal swelling, differential diagnosis of, 267 Scrotal pain, differential diagnosis of, 267 Scrotum, acute, 240 Seborrheic dermatitis, 186 Secondary pulmonary hypertension. See Pulmonary hypertension Sedimentation rate. See Erythrocyte sedimentation rate Seizure disorders definition of, 187 epidemiology and demographics of, 187 etiology of, 187 physical findings and clinical presentation of, 187 Seizures differential diagnosis of generalized, 187 partial, 187 etiology of, 187 treatment of, 187 Selective estrogen receptor modulators (SERM), 148 Selective serotonin reuptake inhibitors (SSRIs), 60 Selegiline (Eldepryl), 153 Senile dementia. See Dementia Senile psychosis. See Dementia Sepsis, 178 Sertraline (Zoloft), 8 Serum albumin, 45 Serum amyloid P[SAP], 7 Serum calcitonin, 210 Serum cholesterol, in cholestatic disorders, 46 Serum creatinine, elevated, 178 Serum ferritin and transferrin saturation, for hemochromatosis, 46 Serum-free PSA, for prostate screening, 166 Serum iron, and total iron-binding capacity patterns, 430t Serum protein electrophoretic patterns, 437f Serum and urinary methylmalonic acid level, 10 Serum vitamin B12 and folic acid, 5 Sevelamer (Renagel), 182 Sex cord stromal tumor. See Ovarian cancer

Sexual dysfunction. See also Erectile dysfunction definition of, 189 diagnosis of, 189 epidemiology and demographics of, 189 etiology of, 189 evaluation of, 381f physical findings and clinical presentation of, 189 treatment of, 189 Sexually transmitted diseases, 267 SGOT. See Aspartate aminotransferase Shingles. See Herpes zoster Short bowel syndrome, 251 Short Michigan Alcoholism Screening Test (SMAST), 4 Shoulder pain, differential diagnosis of, 267 Shy-Drager syndrome, 256, 267 Sibutramine, 140 Sicca syndrome. See Sjögren’s syndrome Sick sinus syndrome, 202 definition of, 190 diagnosis of, 190 epidemiology and demographics of, 190 etiology of, 190 physical findings and clinical presentation of, 190 treatment of, 190 Sigmoidoscopy, 170 Sildenafil, phosphodiesterase inhibitors, 189 Sister Mary Joseph nodule, 149 Sjögren’s syndrome, 68, 191, 240, 247 algorithm for diagnosis of, 382f diagnosis of, 191 epidemiology and demographics of, 191 etiology of, 191 physical findings and clinical presentation of, 191 treatment of, 191 Skilled nursing facility (SNF), 475, 484 Skilled services requirement, 473 Skin cancer. See Squamous cell carcinoma Skull, neoplasms affecting, 29 Sleep apnea, obstructive diagnosis of, 192 epidemiology and demographics of, 192 etiology of, 192 physical findings and clinical presentation of, 192 treatment of, 192 Sleep apnea syndrome, 192, 252 Sleep disorders, 383f–384f, 385 with hypersomnia, 384f Sleep habits, 113b Sleep hygiene measures, 113b Sleep-related GERD, 192 Small bowel obstruction, differential diagnosis of, 268 Sodium, serum, 438 Soluble globular fiber, 63 Spastic colon. See Irritable bowel syndrome Speech pathology, 504 Spinal muscular atrophy (SMA), 8 Spinal stenosis clinical presentation and physical findings of, 194 definition of, 194 diagnosis of, 194 epidemiology and demographics of, 194 etiology of, 194 treatment of, 194 Spiral CT, for diagnosing PE, 172 Splenomegaly clinical approach for, 386f differential diagnosis of, 268 Spondyloarthropathies, 80 Spondylosis. See Spinal stenosis

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Index Sputum for AFB stains and culture, 220 with bacterial respiratory tract infections, 43 gram stain of, 162 Squamous cell carcinoma, 195f definition of, 195 diagnosis of, 195 epidemiology and demographics of, 195 etiology of, 195 physical findings and clinical presentation of, 195 treatment of, 195 Squamous cell carcinoma of vulva. See Vulvar cancer SS. See Sjögren’s syndrome SSRIs, 74 and venlafaxine, 15 ST elevation MI. See Myocardial infarction Staphylococcus aureus, 144 Stasis dermatitis, 196 definition of, 196 diagnosis of, 196 epidemiology and demographics of, 196 etiology of, 196 physical findings and clinical presentation of, 196 treatment of, 196 Stasis, with hyperpigmentation, 196 Status asthmaticuas, 23 Steatohepatitis, differential diagnosis of, 268 Stents coated with paclitaxel, 14 coated with sirolimus, 14 Stevens-Johnson syndrome, 250 Sticky platelet syndrome, 208 Stomach cancer. See Gastric cancer Stones, pigment, with hemolysis and cirrhosis, 42 Stool antigen test, for H. pylori, 154 Streptococcus pneumoniae and Haemophilus influenzae, 44 Streptococcus pyogenes, 144 Streptokinase, thrombolytic agents, 172 Stretta procedure, 82 Stroke, 388 definition of, 197 diagnosis of, 197 differential diagnosis of, 268 epidemiology and demographics of, 197 etiology of, 197 initial considerations for patients with, 199b physical findings and clinical presentation of, 197 treatment of, 199–200 Struvite stones, 225 Subacute conjunctivitis. See Conjunctivitis Subcapital fracture. See Femoral neck fracture Subclinical hyperthyroidism, 107 Subdural hematoma, 201f definition of, 201 diagnosis of, 201 epidemiology and demographics of, 201 etiology of, 201 physical findings and clinical presentation of, 201 treatment of, 201 Subdural space, drainage of blood from, 201 Substance abuse. See Alcoholism Subungual keratosis, 141 Sulfonylurea, 63 Sulfonylureas and meglitinides, 64 Superior vena cava syndrome, 121 Supraspinatus syndrome. See Rotator cuff syndrome Swollen joints, diagnostic approach for, 346f

Symptomatic generalized tonic-clonic seizures, 187 Syncope, 389b caused by orthostatic hypotension, 203 definition of, 202 diagnosis of, 202, 204 epidemiology and demographics of, 202 etiology of, 202–203 evaluation of, 389f physical findings and clinical presentation of, 202 treatment of, 203–204 Synovial aspirate, 86 Synovial fluid analysis, 439 classification and interpretation of, 439t Synovial hypertrophy, 205 Synovium, 169 Syphilis, 443t Systemic lupus erythematosus (SLE), 163

T T scores and Z scores, 146 T3, 439 abnormalities in, 440t T3 resin uptake (T3RU), 439 T4 . See Thyroxine Tachycardia narrow complex, 390 and tachypnea, during asthma, 22 wide complex, 391 Tadalafil, phosphodiesterase inhibitors, 189 Takayasu’s arteritis, 17 Takayasu’s disease, 247 Tamsulosin, 31, 168 Tangier’s disease, 262, 264 Tardive dyskinesia, 204 definition of, 204 differential diagnosis of, 268 epidemiology and demographics of, 204 etiology of, 204 physical findings and clinical presentation of, 204 Tarsal tunnel syndrome, 160 anatomy of, 205f definition of, 205 diagnosis of, 205 epidemiology and demographics of, 205 etiology of, 205 physical findings and clinical presentation of, 205 treatment of, 205 Technetium-99m pertechnetate, 210 Technetium-99m sestamibi, 101 Technetium-99m sulfur colloid scanning, 46 Tegaserod (Zelnorm), 115 Tegretol, 75 Telangiectasia, 195 Telepaque and oragafin, 425 Temporal arteritis. See Giant cell arteritis Tendinitis. See Tendinitis, bicipital Tendinitis, bicipital definition of, 206 diagnosis of, 206 epidemiology and demographics of, 206 etiology of, 206 physical findings and clinical presentation of, 206 treatment of, 206 Tendinopathy, differential diagnosis of, 269 Tenosynovitis, 206 Terazosin, 31, 168 Terbinafine (Lamisil), 142, 211 Testicular germ cell tumor, diagnosis, staging, and risk assessment of, 392f Testicular mass, 392 Testosterone, 440

Thalidomide, with antiangiogenic properties, 133 Thallium, 88 Thiazide diuretics, 101 Thiazides, 91 Thioglitazones (TZDs), 93 Thrombocytopenia, 393b and coagulation factor deficiency, 45 differential diagnosis of, 269 evaluation of, 393f and normal platelet count, 134 Thrombocytosis, 394b diagnosis of, 394f differential diagnosis of, 269 Thrombolytic therapy (streptokinase), 209 Thrombophlebitis, superficial definition of, 207 diagnosis of, 207 epidemiology and demographics of, 207 etiology of, 207 physical findings and clinical presentation of, 207 treatment of, 207 Thrombosis, deep vein diagnosis of, 208 epidemiology and demographics of, 208 etiology of, 208 physical findings and clinical presentation of, 208 treatment of, 208 Thrombotic thrombocytopeic purpura (TIP), 269 Thyroid nodule definition of, 210 diagnosis of, 210 diagnostic evaluation of, 395f epidemiology and demographics of, 210 etiology of, 210 physical findings and clinical presentation of, 210 testing, 369f treatment of, 210 Thyroid-stimulating hormone, 441 Thyrotoxicosis. See Hyperthyroidism Thyroxine, 441 Thyroxine index, free, 425 TIA. See Transient ischemic attack TIBC. See Iron-binding capacity Tic douloureux. See Trigeminal neuralgia Tinea circinata. See Tinea corporis Tinea corporis definition of, 211 diagnosis of, 211 epidemiology and demographics of, 211 etiology of, 211 physical findings and clinical presentation of, 211 treatment of, 211 Tinea pedis, 212f definition of, 212 diagnosis of, 212 epidemiology and demographics of, 212 etiology of, 212 physical findings and clinical presentation of, 212 treatment of, 212 Tinea unguium. See Onychomycosis Tinel’s sign, 205 Tinetti balance and gait evaluation, 529–530 Tinnitus, 397b definition of, 213 diagnosis of, 213 epidemiology and demographics of, 213 etiology of, 213 evaluation of, 397f treatment of, 213 Tirofiban and eptifibatide, 136

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Index Tissue plasminogen activator, 200b Tobacco abuse, 81 Tocainide, 213 Todd’s paralysis, 202 Toe web infection, 212 Tolterodine, 111 Tonsillar and adenoid hypertrophy, 192 Topamax, 74, 159 Topiramate (Topamax), 140, 216 Torticollis. See Dystonia Toxic adenoma, 106 Toxic optic neuropathy, 230t Toxic or drug-induced cataracts. See Cataracts Toxin or drug-induced liver disease, 138 TPA, 172 Trabeculectomy, 85 Tracheomalacia, 116 Transient ischemic attack, 197, 398f definition of, 214 diagnosis of, 214 epidemiology and demographics of, 214 etiology of, 214 hypoglycemia, 214 physical findings and clinical presentation of, 214 and stroke, 131 treatment of, 214 Transitional cell carcinoma (TCC), 32 Transrectal ultrasonography, 166 Transthoracic Doppler echocardiogram, 174 Transurethral incision of prostate (TUIP), 31 Transurethral resection of prostate (TURP), 30, 31 Trauma, 84, 217 with fracture of temporal bone, 29 ice for acute, 37 with valvular rupture, 17 Traumatic cataracts. See Cataracts Traumatic hemolysis, 9 Trazodone, 183 Treadmill exercise, echocardiography with, 13 Tremor, 74 definition of, 74 differential diagnosis of, 74, 270 epidemiology and demographics of, 74 etiology of, 74 physical findings and clinical presentation of, 74 treatment of, 74 Triamcinolone, 217 topical corticosteroid creams, 196 Tricuspid regurgitation (TR), 246 Tricuspid stenosis, 130, 246 Tricyclic antidepressants (TCAs), 60 Trigeminal neuralgia definition of, 216 diagnosis of, 216 epidemiology and demographics of, 216 etiology of, 216 physical findings and clinical presentation of, 216 treatment of, 216 Trigger finger, 217f definition of, 217 diagnosis of, 217 epidemiology and demographics of, 217 etiology of, 217 physical findings and clinical presentation of, 217 treatment of, 217 Triglycerides, 441 Trihexyphenidyl (Artane), 153 Triiodothyronine. See T3 Triptorelin, 167 Trochanteric bursa, injection of, 37

Trochanteric bursitis definition of, 218 diagnosis of, 218 epidemiology and demographics of, 218 etiology of, 218 physical findings and clinical presentation of, 218 treatment of, 218 Trochanteric bursitis syndrome, typical location of pain in, 218f Troponins, serum, 441 Trousseau’s syndrome, 207 TSH. See Thyroid-stimulating hormone Tuberculin tests, interpretation of, 442b Tuberculosis, pulmonary, 121 definition of, 219 diagnosis of, 220 epidemiology and demographics of, 219 etiology of, 219 physical findings and clinical presentation of, 219 treatment of, 220 Tubular necrosis, acute, 178 Tubules, abnormalities of, 266 Tumor within bladder diverticulum, 33 mass lesion, 201 Tumor necrosis factor (TNF), 19, 220 Turcot’s syndrome, 47 Turner’s syndrome, 127 Type-1 DM, 10

U Ulcers arterial, 235 chronic venous stasis, 196 diabetic, 235 differential diagnosis of, 234t foot, 63 gastric, 154 leg, 247f risk factors for arterial, 234 diabetic, 234 pressure, 234 venous, 234 Ultrasonography for detection of gallstones, fatty liver, 46 for dilation of common bile ducts, 46 in identifying aneurysm, 11 Ultraviolet A or B radiation with tanning salons, 28 Unconjugated bilirubin. See Bilirubin, indirect Unconscious patient, approach to, 399f Uncontrollable loss of stool. See Fecal incontinence Uncontrolled loss of urine. See Urinary incontinence Unna boots, for ambulatory patients, 196 Upper motor neurons (UMN), 8 Upper urinary tract, 95 Urea breath test, for H. pylori, 154 Ureteral calculi, management of, 400f Uric acid calculi, 225, 441 Uric acid stones, 225 Urinalysis, 214, 442 for evidence of renal disease, 103 urine C and S, to rule out infection, 31 Urinary abnormalities in renal failure, 179t Urinary incontinence, 110 definition of, 110 diagnosis of, 110 epidemiology and demographics of, 110 etiology of, 110

Urinary incontinence—cont’d physical findings and clinical presentation of, 110 treatment of, 110 Urinary osmolarity, 178 Urinary retention, acute, differential diagnosis of, 270 Urinary sodium and urinary creatinine, 178 Urinary tract infection definition of, 222 diagnosis of, 223 epidemiology and demographics of, 222 management of, 401f treatment of, 223 Urinary tract obstruction, scheme of diagnostic approach to, 402b Urine-5-hydroxyindole-acetic acid, 442 Urine, 443t Urine, red, differential diagnosis of, 270 Urine vanillylmandelic acid, 442 Urokinase, thrombolytic agents, 172 Urolithiasis definition of, 224 diagnosis of, 224 epidemiology and demographics of, 224 etiology of, 224 past medical history significant for, 224b physical findings and clinical presentation of, 224 treatment of, 225 Uropathy diagnostic tests used in obstructive, 402t obstructive, differential diagnosis of, 270 Urticaria, 226f definition of, 226 diagnosis of, 226 epidemiology and demographics of, 226 etiology of, 226 evaluation of, 403f physical findings and clinical presentation of, 226 treatment of, 226 Uterine cancer. See Endometrial cancer Uterine descensus. See Uterine prolapse Uterine prolapse definition of, 227 diagnosis of, 227 epidemiology and demographics of, 227 etiology of, 227 first-degree, 227 physical findings and clinical presentation of, 227 second-degree, 227 third-degree, 227 treatment of, 227 UTI. See Urinary tract infection Utralente, long-acting insulin, 64 Uveitis, 85 Uvulopalatopharyngoplasty, 192

V Vaccines guide to contraindications and precautions to commonly used, 457t–458t guidelines for administering antibodycontaining, 456t VAD. See Vascular dementia Vagal nerve stimulators (VNS), 193 Vagifem, 128, 233 Vaginal apex, 228 Vaginal prolapse, management of, 404f Vaginal spotting, 233 Vaginitis, 223 Vague headache, 201 Valacyclovir (Valtrex), 97

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Index Valproate, 74 Valproic acid, 75 Valvular heart disease, 173 Vancomycin, 170 Vardenafil, 189 Varicella-zoster virus, 97 Varicosities, 205 Vascular dementia, 54 cognitive features of, 55 features of, 54 genetics and family history of, 55 with lewy bodies, 54 main pathologic mechanism of, 55 neuroimaging features of, 55 neurologic features of, 55 pathology of, 55 prevalence of, 54 Vascular endothelial growth factor (VEGF), 48 Vascular malformations, 187 Vascular surgery, in management of chronic venous insufficiency, 196 Vasculature, abnormalities of, 266 Vasodilator medications, 202 Vasodilator treatment with hemodynamic monitoring, 174 Vasomotor symptoms, 127 Ventricular septal defect (VSD), 246 Verapamil, 181 Verrucous carcinoma of vulva. See Vulvar cancer Vertigo, 405b evaluation of, 405f differential diagnosis of, 270 Vesicoureteral reflux, 223 Vibrio vulnificus, 41 Vincristine, 33 doxorubicin (Adriamycin), 132 medications, 88 Viral conjunctivitis, 49f Viral hepatitis, 138 acute, 327f Viral infections, 123 Viral pneumonitis, 121 Visceral cancer, 171 Vision loss acute painful, 271 painless, 271 causes of, 230t chronic, progressive, 271

Vision loss—cont’d definition of, 229 diagnosis of, 230t epidemiology and demographics of, 229 etiology of, 230t monocular, transient, 271 physical findings and clinical presentation of, 229 treatment of, 231 Vitamin B12, 444 Vitreous hemorrhage, causes clouded vision, 229 Voice abuse, 116 leading to hoarseness, 116 Volume depletion, differential diagnosis of, 271 Volume excess, differential diagnosis of, 271 Vomiting, differential diagnosis of, 271 Von Willebrand factor, acquired defect in, 18 V/Q scan, 174 Vulva, advanced postmenopausal atrophy of, 233f Vulvar and vaginal itching, 233 Vulvar cancer, 406 definition of, 232 diagnosis of, 232 epidemiology and demographics of, 232 etiology of, 232 physical findings and clinical presentation of, 232 treatment of, 232 Vulvar intraepithelial neoplasia (VIN), 272 Vulvar lesions, differential diagnosis of, 272 Vulvar sarcoma. See Vulvar cancer Vulvovaginitis, estrogen-deficient cause burning, itching, bleeding, dyspareunia, 127 definition of, 233 diagnosis of, 233 epidemiology and demographics of, 233 etiology of, 233 physical findings and clinical presentation of, 233 treatment of, 233

W Waldenström’s macroglobulinemia, 117, 158 Warfarin anticoagulation with, 24–25 for patients with cardioembolic stroke, 200

Water soluble lubricants, 189 WBC. See Complete blood count Weaver’s bottom (ischial gluteal bursitis), 37 Wegener’s granulomatosis, 22, 163, 179, 247, 254 Weight gain, 407f Weight loss differential diagnosis of, 273 involuntary, 408f Wernicke’s encephalopathy, 244, 263 Wheals. See Urticaria Wheezing during asthma, 22 differential diagnosis of, 273 Whipple’s disease, 244 Whipple’s procedure, 151 White blood count. See Complete blood count White patrolatum, 196 Wilms’ tumor, 176 Wilson’s disease, 45, 68–69, 74, 249 Wiskott-Aldrich syndrome, 269 Wounds definition of, 234 diagnosis of, 234 epidemiology and demographics of, 234 etiology of, 234 physical findings and clinical presentation of, 234 WPW syndrome, 24 Wrist, positive Tinel’s sign on, 39 Writer’s cramp. See Dystonia

X Xerostomia, differential diagnosis of, 273 Xylocaine, 217

Y Yellow nail syndrome, 141

Z Z scores and T scores, 146 Zanamivir, administered via inhaler, 112 Zenker’s diverticulum, 68 Ziehl-Neelsen method, 219 Zollinger-Ellison (Z-E) syndrome, screening for, 155